A Clinical Approach to Children with C3 Glomerulopathy

Overview

Journal Pediatr Nephrol

Specialties Nephrology
Pediatrics

Date 2021 May 18

PMID 34002292

Citations 7

Authors

Marina Vivarelli

Nicole van de Kar

Raffaella Labbadia

Francesca Diomedi-Camassei

Joshua M Thurman

Affiliations

Soon will be listed here.

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.

Citing Articles

Challenges in the Diagnosis and Management of Immune Complex-Mediated Membranoproliferative Glomerulonephritis and Complement 3 Glomerulopathy.

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Complement gene mutations in children with C3 glomerulopathy: do they affect the response to mycophenolate mofetil?.

Gunay N, Dursun I, Gokce I, Akbalik Kara M, Tekcan D, Cicek N Pediatr Nephrol. 2023; 39(5):1435-1446.

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