A clinical approach to children with C3 glomerulopathy.


Journal

Pediatric nephrology (Berlin, Germany)
ISSN: 1432-198X
Titre abrégé: Pediatr Nephrol
Pays: Germany
ID NLM: 8708728

Informations de publication

Date de publication:
03 2022
Historique:
received: 24 01 2021
accepted: 20 04 2021
revised: 28 03 2021
pubmed: 19 5 2021
medline: 16 4 2022
entrez: 18 5 2021
Statut: ppublish

Résumé

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.

Identifiants

pubmed: 34002292
doi: 10.1007/s00467-021-05088-7
pii: 10.1007/s00467-021-05088-7
doi:

Substances chimiques

Complement Inactivating Agents 0

Types de publication

Journal Article Review

Langues

eng

Sous-ensembles de citation

IM

Pagination

521-535

Informations de copyright

© 2021. IPNA.

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Auteurs

Marina Vivarelli (M)

Division of Nephrology and Dialysis, Department of Pediatric Subspecialties, Bambino Gesù Pediatric Hospital IRCCS, Piazza S Onofrio 4, 00165, Rome, Italy. marina.vivarelli@opbg.net.

Nicole van de Kar (N)

Department of Pediatric Nephrology, Amalia Children's Hospital, Radboud University Medical Center, Radboud Institute for Molecular Life Sciences, Nijmegen, The Netherlands.

Raffaella Labbadia (R)

Division of Nephrology and Dialysis, Department of Pediatric Subspecialties, Bambino Gesù Pediatric Hospital IRCCS, Piazza S Onofrio 4, 00165, Rome, Italy.

Francesca Diomedi-Camassei (F)

Pathology Unit, Department of Laboratories, Bambino Gesù Pediatric Hospital IRCCS, Rome, Italy.

Joshua M Thurman (JM)

Department of Medicine, University of Colorado School of Medicine, Anschutz Medical Campus, Aurora, CO, USA.

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