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20.09.2018 | Original Article

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

verfasst von: Johannes Holle, Lena Berenberg-Goßler, Kaiyin Wu, Ortraud Beringer, Florian Kropp, Dominik Müller, Julia Thumfart

Erschienen in: Pediatric Nephrology | Ausgabe 12/2018

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Abstract

Background

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).

Methods

The method used is retrospective analysis of clinical, histological, and genetic characteristics of 14 pediatric patients with MPGN in two medical centers.

Results

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 to end-stage renal disease (ESRD) 6 years after disease onset.

Conclusions

IC-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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Titel: Outcome of membranoproliferative glomerulonephritis and C3-glomerulopathy in children and adolescents
verfasst von: Johannes Holle
Lena Berenberg-Goßler
Kaiyin Wu
Ortraud Beringer
Florian Kropp
Dominik Müller
Julia Thumfart
Publikationsdatum: 20.09.2018
Verlag: Springer Berlin Heidelberg
Erschienen in: Pediatric Nephrology / Ausgabe 12/2018
Print ISSN: 0931-041X
Elektronische ISSN: 1432-198X
DOI: https://doi.org/10.1007/s00467-018-4034-z

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

Abstract

Background

Methods

Results

Conclusions

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Springer Medizin

Abstract

Background

Methods

Results

Conclusions

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