Results 31 to 40 of about 16,816 (256)

Pathology of C3 Glomerulopathy [PDF]

Childhood Kidney Diseases, 2019
C3 glomerulopathy is a renal disorder involving dysregulation of alternative pathway complement activation. In most instances, a membranoproliferative pattern of glomerular injury with a prevalence of C3 deposition is observed by immunofluorescence ...
Su-Jin Shin, Yoonje Seong, Beom Jin Lim
doaj   +1 more source

Dense deposit disease associated with monoclonal gammopathy of undetermined significance. [PDF]

Am J Kidney Dis, 2010
Sethi S, Sukov WR, Zhang Y, Fervenza FC, Lager DJ, Miller DV, Cornell LD, Krishnan SG, Smith RJ.   +8 more
europepmc   +2 more sources

Eculizumab as a treatment for C3 glomerulopathy: a single-center retrospective study

BMC Nephrology, 2023
Background C3 Glomerulopathy (C3G) is a rare glomerular disease caused by dysregulation of the complement pathway. Based on its pathophysiology, treatment with the monoclonal antibody eculizumab targeting complement C5 may be a therapeutic option. Due to
Thomas Welte, Frederic Arnold, Lukas Westermann, Felix A. Rottmann, Martin J. Hug, Elke Neumann-Haefelin, Athina Ganner   +6 more
doaj   +1 more source

Remissions and progression of C3 glomerulopathy

Терапевтический архив, 2022
Aim. To analyze the outcomes of C3-glomerulopathy (C3-GP) and determine the associated factors. Materials and methods. A retrospective single-center study included 60 patients with newly diagnosed C3-GP (with primary C3-GP pC3-GP 82%). Of these, 48 (
Anna V. Karunnaya, Vladimir A. Dobronravov   +1 more
doaj   +1 more source

Complement Factor I Variants in Complement-Mediated Renal Diseases

Frontiers in Immunology, 2022
C3 glomerulopathy (C3G) and atypical hemolytic uremic syndrome (aHUS) are two rare diseases caused by dysregulated activity of the alternative pathway of complement secondary to the presence of genetic and/or acquired factors. Complement factor I (FI) is
Yuzhou Zhang, Renee X. Goodfellow, Nicolo Ghiringhelli Borsa, Hannah C. Dunlop, Stephen A. Presti, Nicole C. Meyer, Dingwu Shao, Sarah M. Roberts, Michael B. Jones, Gabriella R. Pitcher, Amanda O. Taylor, Carla M. Nester, Richard J. H. Smith   +12 more
doaj   +1 more source

Rare Case of C3 Glomerulopathy in a Patient of Type 1 Diabetes Mellitus

Saudi Journal of Kidney Diseases and Transplantation, 2022
Complement component 3 glomerulopathy (C3G) is a recently defined entity comprising dense deposit disease and C3 glomerulonephritis. It is associated with nonrenal diseases such as diabetes mellitus (DM) type 1, ocular drusen, acquired partial ...
Suraj Godara, Karan Kumar Saraf
doaj   +1 more source

Treating C3 glomerulopathy with eculizumab

BMC Nephrology, 2018
Background C3 glomerulopathy (C3G) is a rare, but severe glomerular disease with grim prognosis. The complex pathogenesis is just unfolding, and involves acquired as well as inherited dysregulation of the alternative pathway of the complement cascade ...
Thomas Welte, Frederic Arnold, Julia Kappes, Maximilian Seidl, Karsten Häffner, Carsten Bergmann, Gerd Walz, Elke Neumann-Haefelin   +7 more
doaj   +1 more source

Diverse Clinical Presentations of C3 Dominant Glomerulonephritis

Frontiers in Medicine, 2020
C3 dominant immunofluorescence staining is present in a subset of patients with idiopathic immune complex membranoproliferative glomerulonephritis (iMPGN).
Ramy M. Hanna, Ramy M. Hanna, Jean Hou, Huma Hasnain, Farid Arman, Umut Selamet, Umut Selamet, James Wilson, Samuel Olanrewaju, Jonathan E. Zuckerman, Marina Barsoum, Julie M. Yabu, Ira Kurtz, Ira Kurtz   +13 more
doaj   +1 more source

Gain of function mutant of complement factor B K323E mimics pathogenic C3NeF autoantibodies in convertase assays

Autoimmunity, 2018
Complement convertases are enzymatic complexes, which play a critical role in propagation and amplification of the complement cascade. Under physiological conditions, convertases decay shortly after being formed in either spontaneous or inhibitor-driven ...
Aleksandra Urban, Anna Borowska, Anna Felberg, Lambertus van den Heuvel, Grzegorz Stasiłojć, Elena Volokhina, Marcin Okrój   +6 more
doaj   +1 more source

Regulating complement in the kidney: insights from CFHR5 nephropathy

Disease Models & Mechanisms, 2011
Complement factor H related protein 5 (CFHR5) nephropathy is a monogenic disorder of complement regulation that is endemic in Cyprus. The disease is characterised by haematuria, C3 glomerulonephritis and kidney failure. Its identification suggests a role
Daniel P. Gale, Matthew C. Pickering
doaj   +1 more source