Diagnosis and management of a1-antitrypsin deficiency in Europe: An expert survey
The result's identifiers
Result code in IS VaVaI
<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00064190%3A_____%2F19%3AN0000111" target="_blank" >RIV/00064190:_____/19:N0000111 - isvavai.cz</a>
Result on the web
<a href="http://dx.doi.org/10.1183/23120541.00171-2018" target="_blank" >http://dx.doi.org/10.1183/23120541.00171-2018</a>
DOI - Digital Object Identifier
<a href="http://dx.doi.org/10.1183/23120541.00171-2018" target="_blank" >10.1183/23120541.00171-2018</a>
Alternative languages
Result language
angličtina
Original language name
Diagnosis and management of a1-antitrypsin deficiency in Europe: An expert survey
Original language description
Despite recent improvements, a1-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.
Czech name
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Czech description
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Classification
Type
J<sub>SC</sub> - Article in a specialist periodical, which is included in the SCOPUS database
CEP classification
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OECD FORD branch
30203 - Respiratory systems
Result continuities
Project
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Continuities
N - Vyzkumna aktivita podporovana z neverejnych zdroju
Others
Publication year
2019
Confidentiality
S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů
Data specific for result type
Name of the periodical
ERJ open research
ISSN
2312-0541
e-ISSN
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Volume of the periodical
5
Issue of the periodical within the volume
1
Country of publishing house
GB - UNITED KINGDOM
Number of pages
11
Pages from-to
Article number 00171-2018
UT code for WoS article
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EID of the result in the Scopus database
2-s2.0-85065993868