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Erschienen in: Pediatric Nephrology 3/2022

18.05.2021 | Educational Review

A clinical approach to children with C3 glomerulopathy

verfasst von: Marina Vivarelli, Nicole van de Kar, Raffaella Labbadia, Francesca Diomedi-Camassei, Joshua M. Thurman

Erschienen in: Pediatric Nephrology | Ausgabe 3/2022

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Abstract

C3 glomerulopathy is a relatively new clinical entity that represents a challenge both to diagnose and to treat. As new therapeutic agents that act as complement inhibitors become available, many with an oral formulation, a better understanding of this disease and of the underlying complement dysregulation driving it has become increasingly useful to optimize patient care. Moreover, recent advances in research have clarified the role of complement in other glomerular diseases in which its role was less established, namely in immune-complex membranoproliferative glomerulonephritis (IC-MPGN), ANCA-vasculitis, IgA nephropathy, and idiopathic membranous nephropathy. Complement inhibitors are being studied in adult and adolescent clinical trials for these indications. This review summarizes current knowledge and future perspectives on every aspect of the diagnosis and management of C3 glomerulopathy and elucidates current understanding of the role of complement in this condition and in other glomerular diseases in children. An overview of ongoing trials involving therapeutic agents targeting complement in glomerular diseases is also provided.
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Metadaten
Titel
A clinical approach to children with C3 glomerulopathy
verfasst von
Marina Vivarelli
Nicole van de Kar
Raffaella Labbadia
Francesca Diomedi-Camassei
Joshua M. Thurman
Publikationsdatum
18.05.2021
Verlag
Springer Berlin Heidelberg
Erschienen in
Pediatric Nephrology / Ausgabe 3/2022
Print ISSN: 0931-041X
Elektronische ISSN: 1432-198X
DOI
https://doi.org/10.1007/s00467-021-05088-7

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