Membranoproliferative Glomerulonephritis and C3 Glomerulopathy

2014 ◽  
pp. 189-202
Author(s):  
Daniel P. Gale ◽  
Mared Owen-Casey
Keyword(s):  
Membranoproliferative Glomerulonephritis ◽  
C3 Glomerulopathy

Therapy and outcomes of C3 glomerulopathy and immune-complex membranoproliferative glomerulonephritis

2020 ◽  
Author(s):  
Priyanka Khandelwal ◽  
Swati Bhardwaj ◽  
Geetika Singh ◽  
Aditi Sinha ◽  
Pankaj Hari ◽  
...  
Keyword(s):  
Immune Complex ◽  
Membranoproliferative Glomerulonephritis ◽  
C3 Glomerulopathy

scholarly journals Membranoproliferative glomerulonephritis and C3 glomerulopathy: resolving the confusion

2012 ◽  
Vol 81 (5) ◽  
pp. 434-441 ◽  
Author(s):  
Sanjeev Sethi ◽  
Carla M. Nester ◽  
Richard J.H. Smith
Keyword(s):  
Membranoproliferative Glomerulonephritis ◽  
C3 Glomerulopathy

scholarly journals CFHR5 Genetic Variations and Serum Levels in Patients with Immune-Complex-Mediated Membranoproliferative Glomerulonephritis and C3-Glomerulopathy

2020 ◽  
Author(s):  
Nóra Garam ◽  
Marcell Cserhalmi ◽  
Zoltán Prohászka ◽  
Ágnes Szilágyi ◽  
Nóra Veszeli ◽  
...  
Keyword(s):  
Immune Complex ◽  
Membranoproliferative Glomerulonephritis ◽  
Kidney Diseases ◽  
Alternative Pathway ◽  
Serum Levels ◽  
Genetic Alterations ◽  
Factor H ◽  
Complement Factor ◽  
C3 Glomerulopathy

Abstract Background: Factor H-related-5 (FHR-5) is a member of the complement Factor H protein family. Due to the homology to Factor H, the main complement regulator of the alternative pathway, it may also be implicated in pathomechanism of kidney diseases where Factor H and alternative pathway dysregulation play a role. Here, we report the first observational study on CFHR5 variations along with serum FHR-5 levels in immune-complex-mediated membranoproliferative glomerulonephritis (IC-MPGN) and C3 glomerulopathy (C3G) patients together with the clinical, genetic, complement and follow-up data.Results: 120 patients with a histologically-proven diagnosis of IC-MPGN/C3G were enrolled in the study. FHR-5 serum levels were measured in ELISA, the CFHR5 gene was analyzed by Sanger-sequencing, and selected mutants were studied as recombinant proteins in ELISA and SPR.Eight relevant CFHR5 variations in 14 patients (11.7%) were observed, 5 of them identified as pathogenic for C3G. The FHR-5G278S and FHR-5R356H mutations altered the interaction of FHR-5 with C3b, when compared to the FHR-5WT. Serum FHR-5 levels were lower in patients compared to controls. Low serum FHR-5 concentration at presentation associated with better renal survival during the follow-up period, furthermore, it showed clear association with signs of hypocomplementemia and clinically meaningful clusters.Conclusions: Our observations support the hypothesis that FHR-5 protein and its genetic alterations play a role in the pathogenesis of IC-MPGN/C3G.

Membranoproliferative Glomerulonephritis and C3 Glomerulopathy

2013 ◽  
pp. 31-43
Author(s):  
Agnes B. Fogo ◽  
Arthur H. Cohen ◽  
Robert B. Colvin ◽  
J. Charles Jennette ◽  
Charles E. Alpers
Keyword(s):  
Membranoproliferative Glomerulonephritis ◽  
C3 Glomerulopathy

Membranoproliferative glomerulonephritis and C3 glomerulopathy

2015 ◽  
Author(s):  
Daniel P. Gale ◽  
Terry Cook
Keyword(s):  
Membranoproliferative Glomerulonephritis ◽  
Kidney Biopsy ◽  
Mesangial Cells ◽  
C3 Glomerulopathy ◽  
Thrombotic Microangiopathies ◽  
Dense Deposit ◽  
Wide Range ◽  
Underlying Disorder ◽  
Capillary Walls ◽  
Double Contour

Membranoproliferative glomerulonephritis (MPGN) is synonymous with mesangiocapillary glomerulonephritis and refers to light microscopic appearances of a kidney biopsy in which there are increased mesangial cells and matrix with thickening of the glomerular capillary walls, often with a double contour appearance. MPGN represents morphological appearances caused by a wide range of diseases, most of which are systemic and involve activation of the immune system. It commonly presents as nephrotic syndrome, alternatively with proteinuria, haematuria, and varying degrees of hypertension and renal dysfunction. MPGN was historically characterized into types 1–3 according to the location of immune deposits, but a more useful classification is by whether the underlying disorder results in prominent glomerular immunoglobulin deposition (with secondary complement deposited) or if there is glomerular complement, but scanty or no immunoglobulin deposited. The immunoglobulin group includes MPGN caused by infections, autoimmunity, cryoglobulinaemia, and paraprotein production. The complement group (C3 glomerulopathy) includes dense deposit disease and other complement disorders. Similar light microscopic appearances without deposition of immunoglobulin or complement are sometimes seen in patients with chronic thrombotic microangiopathies. Management and prognosis depend on aetiology.

Membranoproliferative Glomerulonephritis and C3 Glomerulopathy in Children

2021 ◽  
pp. 1-31
Author(s):  
Christoph Licht ◽  
Marina Vivarelli ◽  
Magdalena Riedl Khursigara ◽  
Matthew C. Pickering ◽  
Patrick D. Walker
Keyword(s):  
Membranoproliferative Glomerulonephritis ◽  
C3 Glomerulopathy

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

2018 ◽  
Vol 33 (12) ◽  
pp. 2289-2298 ◽  
Author(s):  
Johannes Holle ◽  
Lena Berenberg-Goßler ◽  
Kaiyin Wu ◽  
Ortraud Beringer ◽  
Florian Kropp ◽  
...  
Keyword(s):  
Children And Adolescents ◽  
Membranoproliferative Glomerulonephritis ◽  
C3 Glomerulopathy
Sign in / Sign up

Export Citation Format

Share Document