H deficiency in two brothers with atypical dense intramembranous deposit disease

M Levy, L Halbwachs–Mecarelli, MC Gubler… - Kidney international, 1986 - Elsevier
H deficiency in two brothers with atypical dense intramembranous deposit disease. We
report an H deficiency in two Algerian brothers who had early–onset glomerulonephritis. In …

Factor H autoantibodies in atypical hemolytic uremic syndrome correlate with CFHR1/CFHR3 deficiency

M Józsi, C Licht, S Strobel, SLH Zipfel… - Blood, The Journal …, 2008 - ashpublications.org
Atypical hemolytic uremic syndrome (aHUS) is a severe renal disease that is associated with
defective complement regulation caused by multiple factors. We previously described the …

Mutations in alternative pathway complement proteins in American patients with atypical hemolytic uremic syndrome

TK Maga, CJ Nishimura, AE Weaver, KL Frees… - Human …, 2010 - Wiley Online Library
Atypical hemolytic uremic syndrome (aHUS) is characterized by acute renal failure,
thrombocytopenia and microangiopathic hemolytic anemia, and occurs with an estimated …

[HTML][HTML] C3 glomerulonephritis: clinicopathological findings, complement abnormalities, glomerular proteomic profile, treatment, and follow-up

S Sethi, FC Fervenza, Y Zhang, L Zand, JA Vrana… - Kidney international, 2012 - Elsevier
C3 glomerulonephritis (C3GN) is a recently described disorder that typically results from
abnormalities in the alternative pathway (AP) of complement. Here, we describe the clinical …

[HTML][HTML] Dense deposit disease and C3 glomerulopathy

TD Barbour, MC Pickering, HT Cook - Seminars in nephrology, 2013 - Elsevier
C3 glomerulopathy refers to those renal lesions characterized histologically by predominant
C3 accumulation within the glomerulus, and pathogenetically by aberrant regulation of the …

Pathogenesis of the C3 glomerulopathies and reclassification of MPGN

AS Bomback, GB Appel - Nature Reviews Nephrology, 2012 - nature.com
Until recently, membranoproliferative glomerulonephritis (MPGN) was clinically classified as
either primary, idiopathic MPGN or as secondary MPGN when an underlying aetiology was …

Atypical haemolytic uraemic syndrome with underlying glomerulopathies. A case series and a review of the literature

L Manenti, E Gnappi, A Vaglio, L Allegri… - Nephrology Dialysis …, 2013 - academic.oup.com
Background Primary or secondary glomerulonephritis has been anecdotally reported in
association with atypical haemolytic uraemic syndrome (aHUS). We here report a series of …

C3 glomerulopathy: understanding an ultra‐rare complement‐mediated renal disease

AK Heiderscheit, JJ Hauer… - American Journal of …, 2022 - Wiley Online Library
Abstract C3 glomerulopathy (C3G) describes a pathologic pattern of injury diagnosed by
renal biopsy. It is characterized by the dominant deposition of the third component of …

[HTML][HTML] C3 glomerulopathy associated with monoclonal Ig is a distinct subtype

A Ravindran, FC Fervenza, RJH Smith, S Sethi - Kidney international, 2018 - Elsevier
Monoclonal immunoglobulins (MIg) may play a causal role in C3 glomerulopathy (C3G) by
impairing regulation of the alternative pathway of complement. Ninety-five patients with C3G …

C3 Glomerulopathy

M Riedl, P Thorner, C Licht - Pediatric Nephrology, 2017 - Springer
Recent advances in our understanding of the disease pathology of membranoproliferative
glomerulonephritis has resulted in its re-classification as complement C3 glomerulopathy …