Diagnosis and management of alpha1-antitrypsin deficiency in Europe: an expert survey

AATD deficiency; Diagnosis; Europe

Detalles Bibliográficos
Autores: Horváth, Ildiko, Canotilho, Maria, Chlumský, Jan, Chorostowska-Wynimko, Joanna, Corda, Luciano, Derom, Eric, Miravitlles Fernández, Marc
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2019
País:España
Institución:Departament de Salut de la Generalitat de Catalunya (DS)
Repositorio:Scientia. Dipòsit d'Informació Digital del Departament de Salut
OAI Identifier:oai:scientiasalut.gencat.cat:11351/4055
Acceso en línea:https://hdl.handle.net/11351/4055
Access Level:acceso abierto
Palabra clave:Malalties congènites - Diagnòstic
Pulmons - Malalties - Europa
DISEASES::Respiratory Tract Diseases::Lung Diseases::alpha 1-Antitrypsin Deficiency
Other subheadings::Other subheadings::/diagnosis
GEOGRAPHICALS::Geographic Locations::Europe
Other subheadings::Other subheadings::Other subheadings::/epidemiology
ENFERMEDADES::enfermedades respiratorias::enfermedades pulmonares::deficiencia de alfa 1-antitripsina
Otros calificadores::Otros calificadores::/diagnóstico
DENOMINACIONES GEOGRÁFICAS::localizaciones geográficas::Europa (continente)
Otros calificadores::Otros calificadores::Otros calificadores::/epidemiología
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spelling Diagnosis and management of alpha1-antitrypsin deficiency in Europe: an expert surveyHorváth, IldikoCanotilho, MariaChlumský, JanChorostowska-Wynimko, JoannaCorda, LucianoDerom, EricMiravitlles Fernández, MarcMalalties congènites - DiagnòsticPulmons - Malalties - EuropaDISEASES::Respiratory Tract Diseases::Lung Diseases::alpha 1-Antitrypsin DeficiencyOther subheadings::Other subheadings::/diagnosisGEOGRAPHICALS::Geographic Locations::EuropeOther subheadings::Other subheadings::Other subheadings::/epidemiologyENFERMEDADES::enfermedades respiratorias::enfermedades pulmonares::deficiencia de alfa 1-antitripsinaOtros calificadores::Otros calificadores::/diagnósticoDENOMINACIONES GEOGRÁFICAS::localizaciones geográficas::Europa (continente)Otros calificadores::Otros calificadores::Otros calificadores::/epidemiologíaAATD deficiency; Diagnosis; EuropeDeficiència d'AATD; Diagnosi; EuropaDeficiencia de AATD; Diagnóstico; EuropaDespite recent improvements, α1-antitrypsin deficiency (AATD) remains a rarely diagnosed and treated condition. To assess the variability of AATD diagnosis/treatment in Europe, and to evaluate clinicians' views on methods to optimise management, specialist AATD clinicians were invited to complete a web-based survey. Surveys were completed by 15 physicians from 14 centres in 13 European countries. All respondents perceived the AATD diagnosis rate to be low in their country; 77% of physicians believed that ∼15% of cases were diagnosed. Low awareness was perceived as the greatest barrier to diagnosis. Spirometry was considered more practical than quantitative computed tomography (QCT) for monitoring AATD patients in clinical practice; QCT was considered more useful in trials. AAT therapy provision was reported to be highly variable: France and Germany were reported to treat the highest proportion (∼60%) of diagnosed patients, in contrast to the UK and Hungary, where virtually no patients receive AAT therapy. Most clinicians supported self-administration and extended dosing intervals to improve convenience of AAT therapy. This survey indicates that AATD diagnosis and management are highly heterogeneous in Europe; European cooperation is essential to generate data to support access to AAT therapy. Improving convenience of AAT therapy is an ongoing objective.European Respiratory SocietyInstitut Català de la Salut[Horváth I] Department of Pulmonology, National Koranyi Institute for Pulmonology in Budapest, Budapest, Hungary. [Canotilho M] Department of Pneumology, Hospital of Santo Andre - Centro Hospitalar de Leiria, Leiria, Portugal. [Chlumský J] Department of Pneumology, Thomayer Hospital Prague, Czech Republic. First Faculty of Medicine, Charles University, Prague, Czech Republic. [Chorostowska-Wynimko J] Department of Genetics and Clinical Immunology, National Institute of Tuberculosis and Lung Diseases, Warsaw, Poland. [Corda L] Department of Internal Medicine, Respiratory Disease Unit, Spedali Civili, Brescia, Italy. [Derom E] Department of Respiratory Medicine, Ghent University Hospital, Ghent, Belgium. [Miravitlles M] Servei de Pneumologia, Hospital Universitari Vall d'Hebron, Barcelona, Spain. CIBER de Enfermedades Respiratorias (CIBERES), Barcelona, Spain.Vall d'Hebron Barcelona Hospital Campus201920192019info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionapplication/pdfhttps://hdl.handle.net/11351/4055Scientiareponame:Scientia. Dipòsit d'Informació Digital del Departament de Salutinstname:Departament de Salut de la Generalitat de Catalunya (DS)InglésERJ Open Research;5(1)https://openres.ersjournals.com/content/5/1/00171-2018Attribution-NonCommercial-NoDerivatives 4.0 Internationalhttp://creativecommons.org/licenses/by-nc-nd/4.0/info:eu-repo/semantics/openAccessoai:scientiasalut.gencat.cat:11351/40552026-06-12T09:38:37Z
dc.title.none.fl_str_mv Diagnosis and management of alpha1-antitrypsin deficiency in Europe: an expert survey
title Diagnosis and management of alpha1-antitrypsin deficiency in Europe: an expert survey
spellingShingle Diagnosis and management of alpha1-antitrypsin deficiency in Europe: an expert survey
Horváth, Ildiko
Malalties congènites - Diagnòstic
Pulmons - Malalties - Europa
DISEASES::Respiratory Tract Diseases::Lung Diseases::alpha 1-Antitrypsin Deficiency
Other subheadings::Other subheadings::/diagnosis
GEOGRAPHICALS::Geographic Locations::Europe
Other subheadings::Other subheadings::Other subheadings::/epidemiology
ENFERMEDADES::enfermedades respiratorias::enfermedades pulmonares::deficiencia de alfa 1-antitripsina
Otros calificadores::Otros calificadores::/diagnóstico
DENOMINACIONES GEOGRÁFICAS::localizaciones geográficas::Europa (continente)
Otros calificadores::Otros calificadores::Otros calificadores::/epidemiología
title_short Diagnosis and management of alpha1-antitrypsin deficiency in Europe: an expert survey
title_full Diagnosis and management of alpha1-antitrypsin deficiency in Europe: an expert survey
title_fullStr Diagnosis and management of alpha1-antitrypsin deficiency in Europe: an expert survey
title_full_unstemmed Diagnosis and management of alpha1-antitrypsin deficiency in Europe: an expert survey
title_sort Diagnosis and management of alpha1-antitrypsin deficiency in Europe: an expert survey
dc.creator.none.fl_str_mv Horváth, Ildiko
Canotilho, Maria
Chlumský, Jan
Chorostowska-Wynimko, Joanna
Corda, Luciano
Derom, Eric
Miravitlles Fernández, Marc
author Horváth, Ildiko
author_facet Horváth, Ildiko
Canotilho, Maria
Chlumský, Jan
Chorostowska-Wynimko, Joanna
Corda, Luciano
Derom, Eric
Miravitlles Fernández, Marc
author_role author
author2 Canotilho, Maria
Chlumský, Jan
Chorostowska-Wynimko, Joanna
Corda, Luciano
Derom, Eric
Miravitlles Fernández, Marc
author2_role author
author
author
author
author
author
dc.contributor.none.fl_str_mv Institut Català de la Salut
[Horváth I] Department of Pulmonology, National Koranyi Institute for Pulmonology in Budapest, Budapest, Hungary. [Canotilho M] Department of Pneumology, Hospital of Santo Andre - Centro Hospitalar de Leiria, Leiria, Portugal. [Chlumský J] Department of Pneumology, Thomayer Hospital Prague, Czech Republic. First Faculty of Medicine, Charles University, Prague, Czech Republic. [Chorostowska-Wynimko J] Department of Genetics and Clinical Immunology, National Institute of Tuberculosis and Lung Diseases, Warsaw, Poland. [Corda L] Department of Internal Medicine, Respiratory Disease Unit, Spedali Civili, Brescia, Italy. [Derom E] Department of Respiratory Medicine, Ghent University Hospital, Ghent, Belgium. [Miravitlles M] Servei de Pneumologia, Hospital Universitari Vall d'Hebron, Barcelona, Spain. CIBER de Enfermedades Respiratorias (CIBERES), Barcelona, Spain.
Vall d'Hebron Barcelona Hospital Campus
dc.subject.none.fl_str_mv Malalties congènites - Diagnòstic
Pulmons - Malalties - Europa
DISEASES::Respiratory Tract Diseases::Lung Diseases::alpha 1-Antitrypsin Deficiency
Other subheadings::Other subheadings::/diagnosis
GEOGRAPHICALS::Geographic Locations::Europe
Other subheadings::Other subheadings::Other subheadings::/epidemiology
ENFERMEDADES::enfermedades respiratorias::enfermedades pulmonares::deficiencia de alfa 1-antitripsina
Otros calificadores::Otros calificadores::/diagnóstico
DENOMINACIONES GEOGRÁFICAS::localizaciones geográficas::Europa (continente)
Otros calificadores::Otros calificadores::Otros calificadores::/epidemiología
topic Malalties congènites - Diagnòstic
Pulmons - Malalties - Europa
DISEASES::Respiratory Tract Diseases::Lung Diseases::alpha 1-Antitrypsin Deficiency
Other subheadings::Other subheadings::/diagnosis
GEOGRAPHICALS::Geographic Locations::Europe
Other subheadings::Other subheadings::Other subheadings::/epidemiology
ENFERMEDADES::enfermedades respiratorias::enfermedades pulmonares::deficiencia de alfa 1-antitripsina
Otros calificadores::Otros calificadores::/diagnóstico
DENOMINACIONES GEOGRÁFICAS::localizaciones geográficas::Europa (continente)
Otros calificadores::Otros calificadores::Otros calificadores::/epidemiología
description AATD deficiency; Diagnosis; Europe
publishDate 2019
dc.date.none.fl_str_mv 2019
2019
2019
dc.type.none.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
format article
status_str publishedVersion
dc.identifier.none.fl_str_mv https://hdl.handle.net/11351/4055
url https://hdl.handle.net/11351/4055
dc.language.none.fl_str_mv Inglés
language_invalid_str_mv Inglés
dc.relation.none.fl_str_mv ERJ Open Research;5(1)
https://openres.ersjournals.com/content/5/1/00171-2018
dc.rights.none.fl_str_mv Attribution-NonCommercial-NoDerivatives 4.0 International
http://creativecommons.org/licenses/by-nc-nd/4.0/
info:eu-repo/semantics/openAccess
rights_invalid_str_mv Attribution-NonCommercial-NoDerivatives 4.0 International
http://creativecommons.org/licenses/by-nc-nd/4.0/
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
dc.publisher.none.fl_str_mv European Respiratory Society
publisher.none.fl_str_mv European Respiratory Society
dc.source.none.fl_str_mv Scientia
reponame:Scientia. Dipòsit d'Informació Digital del Departament de Salut
instname:Departament de Salut de la Generalitat de Catalunya (DS)
instname_str Departament de Salut de la Generalitat de Catalunya (DS)
reponame_str Scientia. Dipòsit d'Informació Digital del Departament de Salut
collection Scientia. Dipòsit d'Informació Digital del Departament de Salut
repository.name.fl_str_mv
repository.mail.fl_str_mv
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score 15.812429

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