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

Autor: Kaiyin Wu, Ortraud Beringer, Florian Kropp, Johannes Holle, Lena Berenberg-Goßler, Julia Thumfart, Dominik N. Müller
Rok vydání: 2018
Předmět:
Nephrology
Male
medicine.medical_specialty
Adolescent
Glomerulonephritis
Membranoproliferative
Thrombomodulin
Complement Pathway
Alternative
Kidney Glomerulus
030232 urology & nephrology
Angiotensin-Converting Enzyme Inhibitors
Disease
030204 cardiovascular system & hematology
Antibodies
Monoclonal
Humanized
03 medical and health sciences
0302 clinical medicine
Glomerulopathy
Internal medicine
Membranoproliferative glomerulonephritis
medicine
Humans
Child
Glucocorticoids
Retrospective Studies
Complement Inactivator Proteins
business.industry
Remission Induction
Complement C3
Eculizumab
Mycophenolic Acid
medicine.disease
Complement system
Patient Outcome Assessment
Treatment Outcome
Pediatrics
Perinatology and Child Health
Alternative complement pathway
Disease Progression
Kidney Failure
Chronic
Female
business
CFHR5
medicine.drug
Follow-Up Studies
Zdroj: Pediatric nephrology (Berlin, Germany). 33(12)
ISSN: 1432-198X
Popis: 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 to end-stage renal disease (ESRD) 6 years after disease onset. 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.
Databáze: OpenAIRE
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