Detail
Článek
Článek online
FT
Medvik - BMČ
  • Je něco špatně v tomto záznamu ?

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,

. 2018 ; 19 (1) : 141. [pub] 20180728

Jazyk angličtina Země Anglie, Velká Británie

Typ dokumentu časopisecké články, multicentrická studie, práce podpořená grantem

Perzistentní odkaz   https://www.medvik.cz/link/bmc19000513

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 1st Faculty of Medicine and Thomayer Hospital Prague Czech Republic

European IPF Registry and Biobank Giessen Germany AGAPLESION Lung Clinic Waldhof Elgershausen Greifenstein Germany

European IPF Registry and Biobank Giessen Germany Competence Center for Rare Pulmonary Diseases Hopital Bichat Paris France

European IPF Registry and Biobank Giessen Germany Department of Clinical and Molecular Biomedicine Università degli Studi di Catania Catania Italy

European IPF Registry and Biobank Giessen Germany Department of Pulmonology Semmelweis University Budapest Hungary

European IPF Registry and Biobank Giessen Germany Department of Thoracic Surgery Vienna University Hospital Vienna Austria

European IPF Registry and Biobank Giessen Germany Hospital Clinico San Carlos Madrid Spain

European IPF Registry and Biobank Giessen Germany Interstitial Lung Disease Unit Royal Brompton Hospital London UK

European IPF Registry and Biobank Giessen Germany Ospedale San Gerardo Monza Italy

European IPF Registry and Biobank Giessen Germany Reference Center for Rare Pulmonary Diseases Centre Hospitalier Universitaire Dijon Bourgogne INSERMU1231 Université Bourgogne Franche Comté Dijon France

European IPF Registry and Biobank Giessen Germany Ruhrlandklinik University Hospital Essen Germany

European IPF Registry and Biobank Giessen Germany Università degli Studi di Napoli Federico 2 Naples Italy

European IPF Registry and Biobank Justus Liebig University Giessen and Johann Wolfgang Goethe University Frankfurt Frankfurt Germany

European IPF Registry and Biobank Klinikstrasse 36 35392 Giessen Germany

Citace poskytuje Crossref.org

000      
00000naa a2200000 a 4500
001      
bmc19000513
003      
CZ-PrNML
005      
20190115123227.0
007      
ta
008      
190107s2018 enk f 000 0|eng||
009      
AR
024    7_
$a 10.1186/s12931-018-0845-5 $2 doi
035    __
$a (PubMed)30055613
040    __
$a ABA008 $b cze $d ABA008 $e AACR2
041    0_
$a eng
044    __
$a enk
100    1_
$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.
245    14
$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,
520    9_
$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 ).
650    _2
$a senioři $7 D000368
650    _2
$a senioři nad 80 let $7 D000369
650    _2
$a biopsie $x mortalita $x trendy $7 D001706
650    _2
$a kohortové studie $7 D015331
650    _2
$a Evropa $x epidemiologie $7 D005060
650    _2
$a ženské pohlaví $7 D005260
650    _2
$a lidé $7 D006801
650    _2
$a idiopatická plicní fibróza $x diagnóza $x mortalita $x patofyziologie $7 D054990
650    _2
$a longitudinální studie $7 D008137
650    _2
$a plíce $x patologie $x patofyziologie $7 D008168
650    _2
$a mužské pohlaví $7 D008297
650    12
$a registrace $7 D012042
650    _2
$a míra přežití $x trendy $7 D015996
655    _2
$a časopisecké články $7 D016428
655    _2
$a multicentrická studie $7 D016448
655    _2
$a práce podpořená grantem $7 D013485
700    1_
$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.
700    1_
$a Tello, Silke $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.
700    1_
$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.
700    1_
$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.
700    1_
$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.
700    1_
$a Maurer, Olga $u European IPF Registry & Biobank (eurIPFreg/bank), Giessen, Germany. AGAPLESION Lung Clinic Waldhof-Elgershausen, Greifenstein, Germany.
700    1_
$a Heinemann, Sabine $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.
700    1_
$a Costabel, Ulrich $u European IPF Registry & Biobank (eurIPFreg/bank), Giessen, Germany. Ruhrlandklinik, University Hospital, Essen, Germany.
700    1_
$a Barbero, María Asunción Nieto $u European IPF Registry & Biobank (eurIPFreg/bank), Giessen, Germany. Hospital Clinico San Carlos, Madrid, Spain.
700    1_
$a Müller, Veronika $u European IPF Registry & Biobank (eurIPFreg/bank), Giessen, Germany. Department of Pulmonology, Semmelweis University, Budapest, Hungary.
700    1_
$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.
700    1_
$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.
700    1_
$a Wells, Athol $u European IPF Registry & Biobank (eurIPFreg/bank), Giessen, Germany. Interstitial Lung Disease Unit, Royal Brompton Hospital, London, UK.
700    1_
$a Vasakova, Martina $u European IPF Registry & Biobank (eurIPFreg/bank), Giessen, Germany. First Faculty of Medicine and Thomayer Hospital, Prague, Czech Republic.
700    1_
$a Pesci, Alberto $u European IPF Registry & Biobank (eurIPFreg/bank), Giessen, Germany. Ospedale San Gerardo, Monza, Italy.
700    1_
$a Sofia, Matteo $u European IPF Registry & Biobank (eurIPFreg/bank), Giessen, Germany. Università degli Studi di Napoli Federico II, Naples, Italy.
700    1_
$a Klepetko, Walter $u European IPF Registry & Biobank (eurIPFreg/bank), Giessen, Germany. Department of Thoracic Surgery, Vienna University Hospital, Vienna, Austria.
700    1_
$a Seeger, Werner $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. Excellence Cluster Cardiopulmonary System (ECCPS), Justus-Liebig University Giessen and Johann Wolfgang Goethe University Frankfurt, Frankfurt, Germany.
700    1_
$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.
700    1_
$a Crestani, Bruno $u European IPF Registry & Biobank (eurIPFreg/bank), Giessen, Germany. Competence Center for Rare Pulmonary Diseases, Hopital Bichat, Paris, France.
773    0_
$w MED00006600 $t Respiratory research $x 1465-993X $g Roč. 19, č. 1 (2018), s. 141
856    41
$u https://pubmed.ncbi.nlm.nih.gov/30055613 $y Pubmed
910    __
$a ABA008 $b sig $c sign $y a $z 0
990    __
$a 20190107 $b ABA008
991    __
$a 20190115123438 $b ABA008
999    __
$a ok $b bmc $g 1364577 $s 1038636
BAS    __
$a 3
BAS    __
$a PreBMC
BMC    __
$a 2018 $b 19 $c 1 $d 141 $e 20180728 $i 1465-993X $m Respiratory research $n Respir Res $x MED00006600
LZP    __
$a Pubmed-20190107

Najít záznam

Citační ukazatele

Nahrávání dat ...

Možnosti archivace

Nahrávání dat ...