Sistema de Salut de Catalunya
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
2019-05-03T10:38:28Z
2019-05-03T10:38:28Z
2019-03-11
AATD deficiency; Diagnosis; Europe
Deficiència d'AATD; Diagnosi; Europa
Deficiencia de AATD; Diagnóstico; Europa
Despite 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.
Article
Published version
English
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
European Respiratory Society
ERJ Open Research;5(1)
https://openres.ersjournals.com/content/5/1/00171-2018
Attribution-NonCommercial-NoDerivatives 4.0 International
http://creativecommons.org/licenses/by-nc-nd/4.0/
Articles científics - HVH [3439]
