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Sökning: id:"swepub:oai:lup.lub.lu.se:e29186a8-d938-488a-b33b-71f4f4e4b983" > Clinical and functi...

  • Miravitlles, MarcVall d'Hebron University Hospital,CIBER Enfermedades Respiratorias (CIBERES) (författare)

Clinical and functional characteristics of individuals with alpha-1 antitrypsin deficiency : EARCO international registry

  • Artikel/kapitelEngelska2022

Förlag, utgivningsår, omfång ...

  • 2022-12-16
  • Springer Science and Business Media LLC,2022

Nummerbeteckningar

  • LIBRIS-ID:oai:lup.lub.lu.se:e29186a8-d938-488a-b33b-71f4f4e4b983
  • https://lup.lub.lu.se/record/e29186a8-d938-488a-b33b-71f4f4e4b983URI
  • https://doi.org/10.1186/s12931-022-02275-4DOI

Kompletterande språkuppgifter

  • Språk:engelska
  • Sammanfattning på:engelska

Ingår i deldatabas

Klassifikation

  • Ämneskategori:art swepub-publicationtype
  • Ämneskategori:ref swepub-contenttype

Anmärkningar

  • 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

Ämnesord och genrebeteckningar

Biuppslag (personer, institutioner, konferenser, titlar ...)

  • Turner, Alice M.University Hospitals Birmingham,University of Birmingham (författare)
  • Torres-Duran, María (författare)
  • Tanash, HananLund 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(Swepub:lu)lung-hat (författare)
  • Rodríguez-García, CarlotaComplejo Hospitalario Universitario de Santiago (författare)
  • López-Campos, José LuisCIBER Enfermedades Respiratorias (CIBERES),University Hospital Virgen del Rocío (författare)
  • Chlumsky, JanCharles University in Prague (författare)
  • Guimaraes, CatarinaHospital da Senhora da Oliveira (författare)
  • Rodríguez-Hermosa, Juan LuisHospital Clinico San Carlos de Madrid,Complutense University of Madrid (författare)
  • Corsico, AngeloUniversity of Pavia,Policlinico San Matteo Pavia Fondazione (författare)
  • Martinez-González, CristinaAsturias General Hospital (författare)
  • Hernández-Pérez, José MaríaCandelaria University Hospital, Tenerife (författare)
  • Bustamante, AnaHospital Sierrallana (författare)
  • Parr, David G.University Hospital Coventry and Warwickshire (författare)
  • Casas-Maldonado, FranciscoHospital Clínico Universitario San Cecilio (författare)
  • Hecimovic, AnaUniversity Hospital of Infectious Diseases (författare)
  • Janssens, WimUniversity Hospitals Leuven,Catholic University of Leuven (författare)
  • Lara, BeatrizUniversity Hospital Coventry and Warwickshire (författare)
  • Barrecheguren, MiriamVall d'Hebron University Hospital (författare)
  • González, CruzUniversity and Polytechnic Hospital la Fe (författare)
  • Stolk, JanLeiden University Medical Centre (författare)
  • Esquinas, CristinaVall d'Hebron University Hospital (författare)
  • Clarenbach, Christian F.University Hospital of Zurich (författare)
  • Vall d'Hebron University HospitalCIBER Enfermedades Respiratorias (CIBERES) (creator_code:org_t)

Sammanhörande titlar

  • Ingår i:Respiratory Research: Springer Science and Business Media LLC23:11465-99211465-993X

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