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C4 nephritic factor in patients with immune-complex-mediated membranoproliferative glomerulonephritis and C3-glomerulopathy
N. Garam, Z. Prohászka, Á. Szilágyi, C. Aigner, A. Schmidt, M. Gaggl, G. Sunder-Plassmann, D. Bajcsi, J. Brunner, A. Dumfarth, D. Cejka, S. Flaschberger, H. Flögelova, Á. Haris, Á. Hartmann, A. Heilos, T. Mueller, K. Rusai, K. Arbeiter, J. Hofer,...
Jazyk angličtina Země Velká Británie
Typ dokumentu časopisecké články, práce podpořená grantem
NLK
BioMedCentral
od 2006-01-12
BioMedCentral Open Access
od 2006
Directory of Open Access Journals
od 2006
Free Medical Journals
od 2006
PubMed Central
od 2006
Europe PubMed Central
od 2006
ProQuest Central
od 2009-01-01
Open Access Digital Library
od 2006-01-01
Open Access Digital Library
od 2006-01-01
Medline Complete (EBSCOhost)
od 2006-01-01
Health & Medicine (ProQuest)
od 2009-01-01
ROAD: Directory of Open Access Scholarly Resources
od 2006
Springer Nature OA/Free Journals
od 2006-12-01
- MeSH
- autoprotilátky imunologie metabolismus MeSH
- dospělí MeSH
- komplement C3 nefritický faktor metabolismus MeSH
- komplement metabolismus MeSH
- lidé MeSH
- membranoproliferativní glomerulonefritida imunologie metabolismus MeSH
- mladiství MeSH
- mladý dospělý MeSH
- nemoci ledvin imunologie metabolismus MeSH
- Check Tag
- dospělí MeSH
- lidé MeSH
- mladiství MeSH
- mladý dospělý MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- práce podpořená grantem MeSH
BACKGROUND: Acquired or genetic abnormalities of the complement alternative pathway are the primary cause of C3glomerulopathy(C3G) but may occur in immune-complex-mediated membranoproliferative glomerulonephritis (IC-MPGN) as well. Less is known about the presence and role of C4nephritic factor(C4NeF) which may stabilize the classical pathway C3-convertase. Our aim was to examine the presence of C4NeF and its connection with clinical features and with other pathogenic factors. RESULTS: One hunfe IC-MPGN/C3G patients were enrolled in the study. C4NeF activity was determined by hemolytic assay utilizing sensitized sheep erythrocytes. Seventeen patients were positive for C4NeF with lower prevalence of renal impairment and lower C4d level, and higher C3 nephritic factor (C3NeF) prevalence at time of diagnosis compared to C4NeF negative patients. Patients positive for both C3NeF and C4NeF had the lowest C3 levels and highest terminal pathway activation. End-stage renal disease did not develop in any of the C4NeF positive patients during follow-up period. Positivity to other complement autoantibodies (anti-C1q, anti-C3) was also linked to the presence of nephritic factors. Unsupervised, data-driven cluster analysis identified a group of patients with high prevalence of multiple complement autoantibodies, including C4NeF. CONCLUSIONS: In conclusion, C4NeF may be a possible cause of complement dysregulation in approximately 10-15% of IC-MPGN/C3G patients.
1st Department of Internal Medicine Semmelweis University Budapest Hungary
1st Department of Internal Medicine University of Szeged Szeged Hungary
1st Department of Pediatrics Semmelweis University Budapest Hungary
6th Department of Medicine Nephrology and Dialysis Wilhelminenspital Vienna Austria
Carol Davila Nephrology Hospital Bucharest Romania
Department of Nephrology Dubrava University Hospital Zagreb Croatia
Department of Nephrology Szent Margit Hospital Budapest Hungary
Department of Pathology of Tartu University Hospital Tartu Estonia
Department of Pediatrics Medical University of Innsbruck Innsbruck Austria
Department of Pediatrics University Hospital and Faculty of Medicine Ostrava Ostrava Czech Republic
Department of Pediatrics University of Debrecen Debrecen Hungary
Department of Pediatrics University of Pécs Pécs Hungary
Department of Pediatrics University of Szeged Szeged Hungary
Dept of Pediatrics Comenius University Bratislava Slovakia
FMC Center of Dialysis Miskolc Hungary
Fundeni Clinical Institute Pediatric Nephrology Department Bucharest Romania
Hospital of Klagenfurt Klagenfurt Austria
Institute of Pathology Faculty of Medicine University of Ljubljana Ljubljana Slovenia
Internal Medicine 4 Section of Nephrology Klinikum Wels Grieskirchen Wels Austria
Nephrology Center Santaros Klinikos Medical Faculty Vilnius University Vilnius Lithuania
Nephrology Clinic 1st Faculty of Medicine Charles University Prague Czech Republic
University Children's Hospital Medical University Sofia Bulgaria
Citace poskytuje Crossref.org
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- $a Garam, Nóra $u Research Laboratory, 3rd Department of Internal Medicine, and MTA-SE Research Group of Immunology and Hematology, Hungarian Academy of Sciences and Semmelweis University, Kútvölgyi St 4, Budapest, H-1125, Hungary.
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- $a C4 nephritic factor in patients with immune-complex-mediated membranoproliferative glomerulonephritis and C3-glomerulopathy / $c N. Garam, Z. Prohászka, Á. Szilágyi, C. Aigner, A. Schmidt, M. Gaggl, G. Sunder-Plassmann, D. Bajcsi, J. Brunner, A. Dumfarth, D. Cejka, S. Flaschberger, H. Flögelova, Á. Haris, Á. Hartmann, A. Heilos, T. Mueller, K. Rusai, K. Arbeiter, J. Hofer, D. Jakab, M. Sinkó, E. Szigeti, C. Bereczki, V. Janko, K. Kelen, GS. Reusz, AJ. Szabó, N. Klenk, K. Kóbor, N. Kojc, M. Knechtelsdorfer, M. Laganovic, AC. Lungu, A. Meglic, R. Rus, T. Kersnik-Levart, E. Macioniene, M. Miglinas, A. Pawłowska, T. Stompór, L. Podracka, M. Rudnicki, G. Mayer, . Romana Rysava, J. Reiterova, M. Saraga, . Tomáš Seeman, J. Zieg, E. Sládková, T. Szabó, A. Capitanescu, S. Stancu, M. Tisljar, K. Galesic, A. Tislér, I. Vainumäe, M. Windpessl, T. Zaoral, G. Zlatanova, D. Csuka,
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- $a BACKGROUND: Acquired or genetic abnormalities of the complement alternative pathway are the primary cause of C3glomerulopathy(C3G) but may occur in immune-complex-mediated membranoproliferative glomerulonephritis (IC-MPGN) as well. Less is known about the presence and role of C4nephritic factor(C4NeF) which may stabilize the classical pathway C3-convertase. Our aim was to examine the presence of C4NeF and its connection with clinical features and with other pathogenic factors. RESULTS: One hunfe IC-MPGN/C3G patients were enrolled in the study. C4NeF activity was determined by hemolytic assay utilizing sensitized sheep erythrocytes. Seventeen patients were positive for C4NeF with lower prevalence of renal impairment and lower C4d level, and higher C3 nephritic factor (C3NeF) prevalence at time of diagnosis compared to C4NeF negative patients. Patients positive for both C3NeF and C4NeF had the lowest C3 levels and highest terminal pathway activation. End-stage renal disease did not develop in any of the C4NeF positive patients during follow-up period. Positivity to other complement autoantibodies (anti-C1q, anti-C3) was also linked to the presence of nephritic factors. Unsupervised, data-driven cluster analysis identified a group of patients with high prevalence of multiple complement autoantibodies, including C4NeF. CONCLUSIONS: In conclusion, C4NeF may be a possible cause of complement dysregulation in approximately 10-15% of IC-MPGN/C3G patients.
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