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Autor
Aigner, Christof 1 Arbeiter, Klaus 1 Bajcsi, Dóra 1 Bereczki, Csaba 1 Brunner, Jürgen 1 Capitanescu, Andrei 1 Cejka, Daniel 1 Csuka, Dorottya 1 Dumfarth, Alexandra 1 Flaschberger, Stefan 1 Flögelova, Hana 1 Gaggl, Martina 1 Galesic, Kresimir 1 Garam, Nóra 1 Haris, Ágnes 1 Hartmann, Ágnes 1 Heilos, Andreas 1 Hofer, Johannes 1 Jakab, Dániel 1 Janko, Viktor 1
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Pracoviště
1st Department of Internal Medicine S... 1 1st Department of Internal Medicine U... 1 1st Department of Pediatrics Semmelwe... 1 Carol Davila Nephrology Hospital Buch... 1 Department of Internal Medicine 4 Nep... 1 Department of Medicine 3 Division of ... 1 Department of Medicine 3 Nephrology T... 1 Department of Nephrology Arterial Hyp... 1 Department of Nephrology Dubrava Univ... 1 Department of Nephrology Hypertension... 1 Department of Nephrology Szent Margit... 1 Department of Nephrology Wilhelminens... 1 Department of Pathology University Ho... 1 Department of Pediatric Nephrology Di... 1 Department of Pediatrics 2nd Faculty ... 1 Department of Pediatrics Comenius Uni... 1 Department of Pediatrics Division of ... 1 Department of Pediatrics Faculty of M... 1 Department of Pediatrics Faculty of M... 1 Department of Pediatrics Faculty of M... 1
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Autor
Aigner, Christof 1 Arbeiter, Klaus 1 Bajcsi, Dóra 1 Bereczki, Csaba 1 Brunner, Jürgen 1 Capitanescu, Andrei 1 Cejka, Daniel 1 Csuka, Dorottya 1 Dumfarth, Alexandra 1 Flaschberger, Stefan 1 Flögelova, Hana 1 Gaggl, Martina 1 Galesic, Kresimir 1 Garam, Nóra 1 Haris, Ágnes 1 Hartmann, Ágnes 1 Heilos, Andreas 1 Hofer, Johannes 1 Jakab, Dániel 1 Janko, Viktor 1
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Pracoviště
1st Department of Internal Medicine S... 1 1st Department of Internal Medicine U... 1 1st Department of Pediatrics Semmelwe... 1 Carol Davila Nephrology Hospital Buch... 1 Department of Internal Medicine 4 Nep... 1 Department of Medicine 3 Division of ... 1 Department of Medicine 3 Nephrology T... 1 Department of Nephrology Arterial Hyp... 1 Department of Nephrology Dubrava Univ... 1 Department of Nephrology Hypertension... 1 Department of Nephrology Szent Margit... 1 Department of Nephrology Wilhelminens... 1 Department of Pathology University Ho... 1 Department of Pediatric Nephrology Di... 1 Department of Pediatrics 2nd Faculty ... 1 Department of Pediatrics Comenius Uni... 1 Department of Pediatrics Division of ... 1 Department of Pediatrics Faculty of M... 1 Department of Pediatrics Faculty of M... 1 Department of Pediatrics Faculty of M... 1
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PubMed Central od 2012
Europe PubMed Central od 2012
Open Access Digital Library od 2012-01-01
Open Access Digital Library od 2015-01-01
Oxford Journals Open Access Collection od 2012-02-01
ROAD: Directory of Open Access Scholarly Resources od 2012
PubMed
32296528
DOI
10.1093/ckj/sfz073
Knihovny.cz E-zdroje
Background: A novel data-driven cluster analysis identified distinct pathogenic patterns in C3-glomerulopathies and immune complex-mediated membranoproliferative glomerulonephritis. Our aim was to replicate these observations in an independent cohort and elucidate disease pathophysiology with detailed analysis of functional complement markers. Methods: A total of 92 patients with clinical, histological, complement and genetic data were involved in the study, and hierarchical cluster analysis was done by Ward method, where four clusters were generated. Results: High levels of sC5b-9 (soluble membrane attack complex), low serum C3 levels and young age at onset (13 years) were characteristic for Cluster 1 with a high prevalence of likely pathogenic variations (LPVs) and C3 nephritic factor, whereas for Cluster 2-which is not reliable because of the small number of cases-strong immunoglobulin G staining, low C3 levels and high prevalence of nephritic syndrome at disease onset were observed. Low plasma sC5b-9 levels, decreased C3 levels and high prevalence of LPV and sclerotic glomeruli were present in Cluster 3, and patients with late onset of the disease (median: 39.5 years) and near-normal C3 levels in Cluster 4. A significant difference was observed in the incidence of end-stage renal disease during follow-up between the different clusters. Patients in Clusters 3-4 had worse renal survival than patients in Clusters 1-2. Conclusions: Our results confirm the main findings of the original cluster analysis and indicate that the observed, distinct pathogenic patterns are replicated in our cohort. Further investigations are necessary to analyse the distinct biological and pathogenic processes in these patient groups.
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Po ukončení testovacího provozu bude odkaz přesměrován adresu produkční verze portálu Medvik.