ArticlePediatric nephrology (Berlin, Germany)2021
Therapy and outcomes of C3 glomerulopathy and immune-complex membranoproliferative glomerulonephritis.
Article 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 26 papers, 1 of them a synthesis that pooled it.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
26 citing papers in PubMed, 1 synthesis or guideline pooled it, 42 citations in OpenAlex.
- Pooled it
- Immunofluorescence-based reclassification of idiopathic membranoproliferative glomerulonephritis: Insights from a single-center cohort.World journal of nephrology · 2026Article
- Time to move to a cluster-based management of C3G/IC-MPGN.Pediatric nephrology (Berlin, Germany) · 2026Article
- Pegcetacoplan in idiopathic and familial pediatric C3 glomerulopathy.Pediatric nephrology (Berlin, Germany) · 2026Observational
- Natural History and Clinical Associations with Long-Term Outcomes in Primary C3 Glomerulopathy and Immune Complex-Mediated Membranoproliferative GN.Clinical journal of the American Society of Nephrology : CJASN · 2026Article
- Article
- Primary membranoproliferative glomerulonephritis: natural history, pathogenesis, and treatment.Frontiers in nephrology · 2026Review
- Successfully treated C3 glomerulopathy in which protein and genetic analyses were useful for diagnosis.CEN case reports · 2025Article
- C3 glomerulopathy in children: a European longitudinal study evaluating outcome.Pediatric nephrology (Berlin, Germany) · 2025Article
- Favorable outcome in children with dense deposit disease with the use of long-term mycophenolate mofetil and high-dose alternate-day steroids.Pediatric nephrology (Berlin, Germany) · 2025Article
- C3 glomerulopathy in children: experience at a resource-limited center.Clinical and experimental pediatrics · 2025Article
- Comparative Analysis of Proteinuria and Longitudinal Outcomes in Immune Complex Membranoproliferative Glomerulonephritis and C3 Glomerulopathy.Kidney international reports · 2025Article
- Clinical characteristics and short term outcomes of childhood immune complex membranoproliferative glomerulonephritis and C3 glomerulopathy: a single centre retrospective study.BMC nephrology · 2025Article
- Challenges in the Diagnosis and Management of Immune Complex-Mediated Membranoproliferative Glomerulonephritis and Complement 3 Glomerulopathy.Kidney international reports · 2025Review
- Clinicopathological characteristics and outcomes of adult patients with idiopathic membranoproliferative glomerulonephritis according to an immunofluorescence-based classification.Journal of nephrology · 2024Article
- Developing Therapies for C3 Glomerulopathy: Report of the Kidney Health Initiative C3 Glomerulopathy Trial Endpoints Work Group.Clinical journal of the American Society of Nephrology : CJASN · 2024Article
- Clinical characteristics and outcomes of immune-complex membranoproliferative glomerulonephritis and C3 glomerulopathy in Japanese children.Pediatric nephrology (Berlin, Germany) · 2024Article
- Apical tubular complement activation and the loss of kidney function in proteinuric kidney diseases.Clinical kidney journal · 2024Review
- Anti-factor B antibodies in atypical hemolytic uremic syndrome.Pediatric nephrology (Berlin, Germany) · 2024Article
- C3G and Ig-MPGN-treatment standard.Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association · 2024Review
Corrections and comments
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Authors and funding
6 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundData on therapy and outcome of dense deposit disease (DDD), C3 glomerulonephritis (C3GN), and immune-complex MPGN (IC-MPGN) in children are limited.
methodsIn this retrospective single-center study from 2007 to 2019, kidney biopsies were reviewed to include patients aged <18-years with C3 glomerulopathy and IC-MPGN. Initial immunosuppression comprised prednisolone, mycophenolate mofetil (n = 51), tacrolimus (n = 11), and/or IV cyclophosphamide (n = 20). Clinicopathological features, response to therapy, and adverse outcome (eGFR
resultsA total of 92 patients were classified as DDD (n = 48, 52.2%), C3GN (n = 26, 28.3%), and IC-MPGN (n = 18, 19.6%) by immunohistochemistry and electron microscopy; 8 patients with DDD were misclassified as IC-MPGN on immunofluorescence. At last follow-up (median 4.3 years), complete or partial remission occurred in 28.5, 36.1, and 16.7% patients with DDD, C3GN, and IC-MPGN, respectively. Serum albumin at onset < 2.5 g/dL (HR = 0.29, P = 0.005) and persistently low serum C3 (HR = 0.34, P = 0.02) were associated with lack of remission. The 5-year kidney survival was 62.6, 85.5, and 88.5% in patients with DDD, C3GN, and IC-MPGN, respectively (log-rank, P = 0.006). Presentation as rapidly progressive GN (HR = 11.2, P < 0.001), age > 10 years at onset (HR = 4.0, P = 0.004), and DDD (HR = 4.2, P = 0.02) were independently associated with adverse outcome; achieving remission was protective (HR = 0.04; P < 0.001).
conclusionOutcome in patients with C3 glomerulopathy and IC-MPGN was unsatisfactory, and only a small proportion of patients achieved complete or partial remission. Patients with DDD were more likely to present with rapidly progressive GN and were at higher risk of adverse outcomes, including kidney failure.
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