Outcome of membranoproliferative glomerulonephritis and C3-glomerulopathy in children and adolescents

Pediatric Nephrology : Journal of the International Pediatric Nephrology Association
Johannes HolleJulia Thumfart

Abstract

Membranoproliferative 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). The method used is retrospective analysis of clinical, histological, and genetic characteristics of 14 pediatric patients with MPGN in two medical centers. Mean 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 t...Continue Reading

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Citations

Sep 5, 2020·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Priyanka KhandelwalArvind Bagga
Jan 10, 2020·Acta Clinica Belgica·Fatma YazılıtaşMehmet Bülbül
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Jul 6, 2020·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Xu ZhangSuxia Wang
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