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Clinical and functi...
Clinical and functional characteristics of individuals with alpha-1 antitrypsin deficiency : EARCO international registry
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- Miravitlles, Marc (author)
- Vall d'Hebron University Hospital,CIBER Enfermedades Respiratorias (CIBERES)
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- Turner, Alice M. (author)
- University of Birmingham,University Hospitals Birmingham
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Torres-Duran, María (author)
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- Tanash, Hanan (author)
- Lund University,Lunds universitet,Lungmedicin, allergologi och palliativ medicin,Sektion II,Institutionen för kliniska vetenskaper, Lund,Medicinska fakulteten,Alfa-1-antritrypsinbrist,Forskargrupper vid Lunds universitet,Respiratory Medicine, Allergology, and Palliative Medicine,Section II,Department of Clinical Sciences, Lund,Faculty of Medicine,Alpha-1-antitrypsin deficiency,Lund University Research Groups,Skåne University Hospital
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- Rodríguez-García, Carlota (author)
- Complejo Hospitalario Universitario de Santiago
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- López-Campos, José Luis (author)
- University Hospital Virgen del Rocío,CIBER Enfermedades Respiratorias (CIBERES)
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- Chlumsky, Jan (author)
- Charles University in Prague
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- Guimaraes, Catarina (author)
- Hospital da Senhora da Oliveira
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- Rodríguez-Hermosa, Juan Luis (author)
- Hospital Clinico San Carlos de Madrid,Complutense University of Madrid
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- Corsico, Angelo (author)
- Policlinico San Matteo Pavia Fondazione,University of Pavia
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- Martinez-González, Cristina (author)
- Asturias General Hospital
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- Hernández-Pérez, José María (author)
- Candelaria University Hospital, Tenerife
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- Bustamante, Ana (author)
- Hospital Sierrallana
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- Parr, David G. (author)
- University Hospital Coventry and Warwickshire
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- Casas-Maldonado, Francisco (author)
- Hospital Clínico Universitario San Cecilio
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- Hecimovic, Ana (author)
- University Hospital of Infectious Diseases
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- Janssens, Wim (author)
- University Hospitals Leuven,Catholic University of Leuven
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- Lara, Beatriz (author)
- University Hospital Coventry and Warwickshire
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- Barrecheguren, Miriam (author)
- Vall d'Hebron University Hospital
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- González, Cruz (author)
- University and Polytechnic Hospital la Fe
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- Stolk, Jan (author)
- Leiden University Medical Centre
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- Esquinas, Cristina (author)
- Vall d'Hebron University Hospital
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- Clarenbach, Christian F. (author)
- University Hospital of Zurich
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(creator_code:org_t)
- 2022-12-16
- 2022
- English.
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In: Respiratory Research. - : Springer Science and Business Media LLC. - 1465-9921 .- 1465-993X. ; 23:1
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http://dx.doi.org/10... (free)
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Abstract
Subject headings
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- Background: Alpha-1 antitrypsin deficiency (AATD) is a rare disease that is associated with an increased risk of pulmonary emphysema. The European AATD Research Collaboration (EARCO) international registry was founded with the objective of characterising the individuals with AATD and investigating their natural history. Methods: The EARCO registry is an international, observational and prospective study of individuals with AATD, defined as AAT serum levels < 11 μM and/or proteinase inhibitor genotypes PI*ZZ, PI*SZ and compound heterozygotes or homozygotes of other rare deficient variants. We describe the characteristics of the individuals included from February 2020 to May 2022. Results: A total of 1044 individuals from 15 countries were analysed. The most frequent genotype was PI*ZZ (60.2%), followed by PI*SZ (29.2%). Among PI*ZZ patients, emphysema was the most frequent lung disease (57.2%) followed by COPD (57.2%) and bronchiectasis (22%). Up to 76.4% had concordant values of FEV1(%) and KCO(%). Those with impairment in FEV1(%) alone had more frequently bronchiectasis and asthma and those with impairment in KCO(%) alone had more frequent emphysema and liver disease. Multivariate analysis showed that advanced age, male sex, exacerbations, increased blood platelets and neutrophils, augmentation and lower AAT serum levels were associated with worse FEV1(%). Conclusions: EARCO has recruited > 1000 individuals with AATD from 15 countries in its first 2 years. Baseline cross sectional data provide relevant information about the clinical phenotypes of the disease, the patterns of functional impairment and factors associated with poor lung function. Trial registrationwww.clinicaltrials.gov
Subject headings
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Lungmedicin och allergi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Respiratory Medicine and Allergy (hsv//eng)
Keyword
- Alpha-1 antitrypsin
- Phenotypes
- Registry
Publication and Content Type
- art (subject category)
- ref (subject category)
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- By the author/editor
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Miravitlles, Mar ...
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Turner, Alice M.
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Torres-Duran, Ma ...
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Tanash, Hanan
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Rodríguez-García ...
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López-Campos, Jo ...
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show more...
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Chlumsky, Jan
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Guimaraes, Catar ...
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Rodríguez-Hermos ...
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Corsico, Angelo
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Martinez-Gonzále ...
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Hernández-Pérez, ...
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Bustamante, Ana
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Parr, David G.
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Casas-Maldonado, ...
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Hecimovic, Ana
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Janssens, Wim
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Lara, Beatriz
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Barrecheguren, M ...
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González, Cruz
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Stolk, Jan
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Esquinas, Cristi ...
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Clarenbach, Chri ...
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- About the subject
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- MEDICAL AND HEALTH SCIENCES
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MEDICAL AND HEAL ...
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and Clinical Medicin ...
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and Respiratory Medi ...
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Respiratory Rese ...
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Lund University