Results 71 to 80 of about 216,693 (188)
C3-Glomerulopathy Autoantibodies Mediate Distinct Effects on Complement C3- and C5-Convertases
C3 glomerulopathy (C3G) is a severe kidney disease, which is caused by defective regulation of the alternative complement pathway. Disease pathogenesis is heterogeneous and is caused by both autoimmune and genetic factors.
Fei Zhao +13 more
doaj +1 more source
Abstract Background and Purpose Overactivation of the alternative pathway (AP) underlies several diseases. Iptacopan is an oral, first‐in‐class, highly potent specific inhibitor of factor B, a key AP protease. Experimental Approach The analysis included data from two phase 1 randomised, volunteer‐blinded, placebo‐controlled studies: Study 1, a single ...
Irina Baltcheva +5 more
wiley +1 more source
Glomerular capillary microaneurysms (GCMs) were most frequently observed in proliferative glomerulonephritis with monoclonal immunoglobulin G deposits (PGNMID) among MPGN‐pattern glomerular diseases, but were not restricted to this entity. These lesions were characterized by exclusive IgG3‐dominant glomerular deposition, irrespective of light‐chain ...
Akiko Mii +11 more
wiley +1 more source
Current concepts in C3 glomerulopathy
Complement component 3 glomerulopathy (C3G) is a recently defined entity comprising of dense deposit disease and C3 glomerulonephritis. The key histological feature is the presence of isolated C3 deposits without immunoglobulins. Often masqueradng as some of the common glomerulonephritides this is a prototype disorder occurring from dysregulated ...
Thomas, S. +4 more
openaire +2 more sources
Circulating Complement Levels and C3 Glomerulopathy [PDF]
The complement system is an essential part of innate immunity acting as a first-line defense against infection and provides an interface between innate and adaptive immunity ([1][1],[2][2]). It consists of a network of soluble (fluid phase) and cell membrane proteins (solid phase).
Fernando C, Fervenza, Sanjeev, Sethi
openaire +2 more sources
ABSTRACT The coexistence of primary phospholipase A2 receptor positive membranous nephropathy and AA amyloidosis in a patient with chronic hepatitis B is an exceedingly rare triad presenting a profound diagnostic and therapeutic challenge. A 38‐year‐old Ethiopian man with nephrotic syndrome and chronic hepatitis B had dual pathology on renal biopsy ...
Betelhem Abreham +4 more
wiley +1 more source
Predictors of poor kidney outcome in children with C3 glomerulopathy
Background C3 glomerulopathy (C3G) is characterized by heterogeneous clinical presentation, outcome, and predominant C3 accumulation in glomeruli without significant IgG. There is scarce outcome data regarding childhood C3G.
Cicek, Neslihan +28 more
core +1 more source
Four cases of C3 glomerulopathy in children and literature review [PDF]
Objective To investigate the clinical phenotypes, pathological characteristics, genetic variants, and treatment outcomes in children with C3 glomerulopathy (C3G), and to provide evidence for precise diagnosis and management.
LI Huarong, CHEN Chaoying, TU Juan, LIN Tiantian, WANG Nannan
doaj +1 more source
Local complement production by pulmonary artery adventitial fibroblasts, activated intracellularly by CFD and CFB and extracellularly by GZMK+ CD8 T cells, and its secretion in soluble form and within EVs promotes macrophage and T cell chemotaxis and activation.
Hui Zhang +9 more
wiley +1 more source
C3 glomerulopathy — understanding a rare complement-driven renal disease
\ua9 2019, Springer Nature Limited. The C3 glomerulopathies are a group of rare kidney diseases characterized by complement dysregulation occurring in the fluid phase and in the glomerular microenvironment, which results in prominent complement C3 ...
Van der Vlag J +17 more
core +6 more sources

