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The European IPF registry (eurIPFreg): baseline characteristics and survival of patients with idiopathic pulmonary fibrosis
A. Guenther, E. Krauss, S. Tello, J. Wagner, B. Paul, S. Kuhn, O. Maurer, S. Heinemann, U. Costabel, MAN. Barbero, V. Müller, P. Bonniaud, C. Vancheri, A. Wells, M. Vasakova, A. Pesci, M. Sofia, W. Klepetko, W. Seeger, F. Drakopanagiotakis, B. Crestani,
Jazyk angličtina Země Anglie, Velká Británie
Typ dokumentu časopisecké články, multicentrická studie, práce podpořená grantem
NLK
BioMedCentral
od 2000-01-04
BioMedCentral Open Access
od 2000
Directory of Open Access Journals
od 2000
Free Medical Journals
od 2000
PubMed Central
od 2000
Europe PubMed Central
od 2000
ProQuest Central
od 2009-01-01
Open Access Digital Library
od 2000-06-01
Open Access Digital Library
od 2000-01-01
Open Access Digital Library
od 2000-01-01
Medline Complete (EBSCOhost)
od 2000-01-01
Health & Medicine (ProQuest)
od 2009-01-01
ROAD: Directory of Open Access Scholarly Resources
od 2000
Springer Nature OA/Free Journals
od 2000-04-01
- MeSH
- biopsie mortalita trendy MeSH
- idiopatická plicní fibróza diagnóza mortalita patofyziologie MeSH
- kohortové studie MeSH
- lidé MeSH
- longitudinální studie MeSH
- míra přežití trendy MeSH
- plíce patologie patofyziologie MeSH
- registrace * MeSH
- senioři nad 80 let MeSH
- senioři MeSH
- Check Tag
- lidé MeSH
- mužské pohlaví MeSH
- senioři nad 80 let MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- multicentrická studie MeSH
- práce podpořená grantem MeSH
- Geografické názvy
- Evropa epidemiologie MeSH
BACKGROUND: Since 2009, IPF patients across Europe are recruited into the eurIPFreg, providing epidemiological data and biomaterials for translational research. METHODS: The registry data are based on patient and physician baseline and follow-up questionnaires, comprising 1700 parameters. The mid- to long-term objectives of the registry are to provide clues for a better understanding of IPF phenotype sub-clusters, triggering factors and aggravating conditions, regional and environmental characteristics, and of disease behavior and management. RESULTS: This paper describes baseline data of 525 IPF subjects recruited from 11/2009 until 10/2016. IPF patients had a mean age of 68.1 years, and seeked medical advice due to insidious dyspnea (90.1%), fatigue (69.2%), and dry coughing (53.2%). A surgical lung biopsy was performed in 32% in 2009, but in only 8% of the cases in 2016, possibly due to increased numbers of cryobiopsy. At the time of inclusion in the eurIPFreg, FVC was 68.4% ± 22.6% of predicted value, DLco ranged at 42.1% ± 17.8% of predicted value (mean value ± SD). Signs of pulmonary hypertension were found in 16.8%. Steroids, immunosuppressants and N-Acetylcysteine declined since 2009, and were replaced by antifibrotics, under which patients showed improved survival (p = 0.001). CONCLUSIONS: Our data provide important insights into baseline characteristics, diagnostic and management changes as well as outcome data in European IPF patients over time. TRIAL REGISTRATION: The eurIPFreg and eurIPFbank are listed in ClinicalTrials.gov( NCT02951416 ).
European IPF Registry and Biobank Giessen Germany
European IPF Registry and Biobank Giessen Germany Hospital Clinico San Carlos Madrid Spain
European IPF Registry and Biobank Giessen Germany Ospedale San Gerardo Monza Italy
European IPF Registry and Biobank Giessen Germany Ruhrlandklinik University Hospital Essen Germany
European IPF Registry and Biobank Klinikstrasse 36 35392 Giessen Germany
Citace poskytuje Crossref.org
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- $a Guenther, Andreas $u European IPF Registry & Biobank (eurIPFreg/bank), Giessen, Germany. andreas.guenther@innere.med.uni-giessen.de. Universities of Giessen and Marburg Lung Center (UGMLC), Member of the German Center for Lung Research (DZL), Giessen, Germany. andreas.guenther@innere.med.uni-giessen.de. Excellence Cluster Cardiopulmonary System (ECCPS), Justus-Liebig University Giessen and Johann Wolfgang Goethe University Frankfurt, Frankfurt, Germany. andreas.guenther@innere.med.uni-giessen.de. AGAPLESION Lung Clinic Waldhof-Elgershausen, Greifenstein, Germany. andreas.guenther@innere.med.uni-giessen.de. Clinical Research Unit "Pulmonary Fibrosis", University of Giessen and Marburg Lung Center (UGMLC), German Center for Lung Research (DZL), European IPF Registry (eurIPFreg), Klinikstrasse 36, 35392, Giessen, Germany. andreas.guenther@innere.med.uni-giessen.de.
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- $a The European IPF registry (eurIPFreg): baseline characteristics and survival of patients with idiopathic pulmonary fibrosis / $c A. Guenther, E. Krauss, S. Tello, J. Wagner, B. Paul, S. Kuhn, O. Maurer, S. Heinemann, U. Costabel, MAN. Barbero, V. Müller, P. Bonniaud, C. Vancheri, A. Wells, M. Vasakova, A. Pesci, M. Sofia, W. Klepetko, W. Seeger, F. Drakopanagiotakis, B. Crestani,
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- $a BACKGROUND: Since 2009, IPF patients across Europe are recruited into the eurIPFreg, providing epidemiological data and biomaterials for translational research. METHODS: The registry data are based on patient and physician baseline and follow-up questionnaires, comprising 1700 parameters. The mid- to long-term objectives of the registry are to provide clues for a better understanding of IPF phenotype sub-clusters, triggering factors and aggravating conditions, regional and environmental characteristics, and of disease behavior and management. RESULTS: This paper describes baseline data of 525 IPF subjects recruited from 11/2009 until 10/2016. IPF patients had a mean age of 68.1 years, and seeked medical advice due to insidious dyspnea (90.1%), fatigue (69.2%), and dry coughing (53.2%). A surgical lung biopsy was performed in 32% in 2009, but in only 8% of the cases in 2016, possibly due to increased numbers of cryobiopsy. At the time of inclusion in the eurIPFreg, FVC was 68.4% ± 22.6% of predicted value, DLco ranged at 42.1% ± 17.8% of predicted value (mean value ± SD). Signs of pulmonary hypertension were found in 16.8%. Steroids, immunosuppressants and N-Acetylcysteine declined since 2009, and were replaced by antifibrotics, under which patients showed improved survival (p = 0.001). CONCLUSIONS: Our data provide important insights into baseline characteristics, diagnostic and management changes as well as outcome data in European IPF patients over time. TRIAL REGISTRATION: The eurIPFreg and eurIPFbank are listed in ClinicalTrials.gov( NCT02951416 ).
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- $a Krauss, Ekaterina $u European IPF Registry & Biobank (eurIPFreg/bank), Giessen, Germany. Universities of Giessen and Marburg Lung Center (UGMLC), Member of the German Center for Lung Research (DZL), Giessen, Germany.
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- $a Wagner, Jasmin $u European IPF Registry & Biobank (eurIPFreg/bank), Giessen, Germany. Universities of Giessen and Marburg Lung Center (UGMLC), Member of the German Center for Lung Research (DZL), Giessen, Germany.
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- $a Paul, Bettina $u European IPF Registry & Biobank (eurIPFreg/bank), Giessen, Germany. Universities of Giessen and Marburg Lung Center (UGMLC), Member of the German Center for Lung Research (DZL), Giessen, Germany.
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- $a Kuhn, Stefan $u European IPF Registry & Biobank (eurIPFreg/bank), Giessen, Germany. Universities of Giessen and Marburg Lung Center (UGMLC), Member of the German Center for Lung Research (DZL), Giessen, Germany.
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- $a Maurer, Olga $u European IPF Registry & Biobank (eurIPFreg/bank), Giessen, Germany. AGAPLESION Lung Clinic Waldhof-Elgershausen, Greifenstein, Germany.
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- $a Bonniaud, Philippe $u European IPF Registry & Biobank (eurIPFreg/bank), Giessen, Germany. Reference Center for Rare Pulmonary Diseases, Centre Hospitalier Universitaire Dijon-Bourgogne, INSERMU1231, Université Bourgogne/Franche-Comté, Dijon, France.
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- $a Vancheri, Carlo $u European IPF Registry & Biobank (eurIPFreg/bank), Giessen, Germany. Department of Clinical and Molecular Biomedicine, Università degli Studi di Catania, Catania, Italy.
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- $a Wells, Athol $u European IPF Registry & Biobank (eurIPFreg/bank), Giessen, Germany. Interstitial Lung Disease Unit, Royal Brompton Hospital, London, UK.
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- $a Drakopanagiotakis, Fotios $u European IPF Registry & Biobank (eurIPFreg/bank), Giessen, Germany. Universities of Giessen and Marburg Lung Center (UGMLC), Member of the German Center for Lung Research (DZL), Giessen, Germany.
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