European Respiratory Society statement: diagnosis and treatment of pulmonary disease in α1-antitrypsin deficiency

Result code in IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00216208%3A11150%2F17%3A10366945" target="_blank" >RIV/00216208:11150/17:10366945 - isvavai.cz</a>

Result on the web

<a href="http://dx.doi.org/10.1183/13993003.00610-2017" target="_blank" >http://dx.doi.org/10.1183/13993003.00610-2017</a>

DOI - Digital Object Identifier

<a href="http://dx.doi.org/10.1183/13993003.00610-2017" target="_blank" >10.1183/13993003.00610-2017</a>

Result language

angličtina

Original language name

European Respiratory Society statement: diagnosis and treatment of pulmonary disease in α1-antitrypsin deficiency

Original language description

α1-antitrypsin deficiency (AATD) is the most common hereditary disorder in adults. It is associated with an increased risk of developing pulmonary emphysema and liver disease. The pulmonary emphysema in AATD is strongly linked to smoking, but even a proportion of never-smokers develop progressive lung disease. A large proportion of individuals affected remain undiagnosed and therefore without access to appropriate care and treatment. The most recent international statement on AATD was published by the American Thoracic Society and the European Respiratory Society in 2003. Since then there has been a continuous development of novel, more accurate and less expensive genetic diagnostic methods. Furthermore, new outcome parameters have been developed and validated for use in clinical trials and a new series of observational and randomised clinical trials have provided more evidence concerning the efficacy and safety of augmentation therapy, the only specific treatment available for the pulmonary disease associated with AATD. As AATD is a rare disease, it is crucial to organise national and international registries and collect information prospectively about the natural history of the disease. Management of AATD patients must be supervised by national or regional expert centres and inequalities in access to therapies across Europe should be addressed.

Czech name

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Czech description

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Type

J_imp - Article in a specialist periodical, which is included in the Web of Science database

CEP classification

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OECD FORD branch

30203 - Respiratory systems

Project

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Continuities

I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Publication year

2017

Confidentiality

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Name of the periodical

European Respiratory Journal

ISSN

0903-1936

e-ISSN

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Volume of the periodical

50

Issue of the periodical within the volume

5

Country of publishing house

GB - UNITED KINGDOM

Number of pages

24

Pages from-to

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UT code for WoS article

000422739100004

EID of the result in the Scopus database

2-s2.0-85039075322