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Ongoing research in Europe: Alpha One International Registry (AIR) objectives and development.

Stockley, R A; Luisetti, M; Miravitlles, M; Piitulainen, Eeva LU and Fernandez, P (2007) In European Respiratory Journal 29(3). p.582-586
Abstract
In 1997, the World Health Organization recommended establishing an international registry of alpha(1)-antitrypsin deficiency. The objective of the present article is to describe the Organisation of an international network of registries, the Alpha One International Registry (AIR), and the processes of enrolling and entering data. By the end of 2005, the registry included individuals from 21 countries (from four continents). The inclusion criterion was either phenotypes PiZZ, PiSZ or other severely deficient variants. Demographic and clinical information have been collected by a standardised questionnaire, translated for each country. Data are transferred to the AIR database at the Dept of Respiratory Medicine, University Hospital, Malmo,... (More)
In 1997, the World Health Organization recommended establishing an international registry of alpha(1)-antitrypsin deficiency. The objective of the present article is to describe the Organisation of an international network of registries, the Alpha One International Registry (AIR), and the processes of enrolling and entering data. By the end of 2005, the registry included individuals from 21 countries (from four continents). The inclusion criterion was either phenotypes PiZZ, PiSZ or other severely deficient variants. Demographic and clinical information have been collected by a standardised questionnaire, translated for each country. Data are transferred to the AIR database at the Dept of Respiratory Medicine, University Hospital, Malmo, Sweden, either by e-mail or via two web-enabled questionnaires in HTML. All data are merged and checked for consistency and missing values. Collection of data started in 1999 and, by September 2005, data on 2,150 individual patients (1,180 male) had been submitted. Of these, 1,855 (84%) have phenotype PiZ, 181 (8%) PiSZ and 114 (5%) other rare Pi phenotypes. The mean age at inclusion was 49.8 yrs (SD=13.3) and the majority were index cases (64.1%). The Alpha One International Registry is the largest specific alpha(1)-antitrypsin deficiency registry, fulfilling a major World Health Organization recommendation. The success related to the convergence of national registries into a common database creating a unique registry beyond geographic boundaries and encompassing alpha(1)-antitrypsin deficiency from various ethnic groups. (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
epidemiology, emphysema, augmentation therapy, registries, prevalence, chronic obstructive pulmonary disease
in
European Respiratory Journal
volume
29
issue
3
pages
582 - 586
publisher
Eur Respiratory Soc
external identifiers
  • wos:000244967500026
  • scopus:33847750273
ISSN
1399-3003
DOI
10.1183/​09031936.00053606
language
English
LU publication?
yes
id
f0ff1f6f-4519-4926-96a7-ba6f62bf1e5f (old id 166921)
alternative location
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=17329492&dopt=Abstract
date added to LUP
2007-07-11 14:20:20
date last changed
2017-11-12 03:25:23
@article{f0ff1f6f-4519-4926-96a7-ba6f62bf1e5f,
  abstract     = {In 1997, the World Health Organization recommended establishing an international registry of alpha(1)-antitrypsin deficiency. The objective of the present article is to describe the Organisation of an international network of registries, the Alpha One International Registry (AIR), and the processes of enrolling and entering data. By the end of 2005, the registry included individuals from 21 countries (from four continents). The inclusion criterion was either phenotypes PiZZ, PiSZ or other severely deficient variants. Demographic and clinical information have been collected by a standardised questionnaire, translated for each country. Data are transferred to the AIR database at the Dept of Respiratory Medicine, University Hospital, Malmo, Sweden, either by e-mail or via two web-enabled questionnaires in HTML. All data are merged and checked for consistency and missing values. Collection of data started in 1999 and, by September 2005, data on 2,150 individual patients (1,180 male) had been submitted. Of these, 1,855 (84%) have phenotype PiZ, 181 (8%) PiSZ and 114 (5%) other rare Pi phenotypes. The mean age at inclusion was 49.8 yrs (SD=13.3) and the majority were index cases (64.1%). The Alpha One International Registry is the largest specific alpha(1)-antitrypsin deficiency registry, fulfilling a major World Health Organization recommendation. The success related to the convergence of national registries into a common database creating a unique registry beyond geographic boundaries and encompassing alpha(1)-antitrypsin deficiency from various ethnic groups.},
  author       = {Stockley, R A and Luisetti, M and Miravitlles, M and Piitulainen, Eeva and Fernandez, P},
  issn         = {1399-3003},
  keyword      = {epidemiology,emphysema,augmentation therapy,registries,prevalence,chronic obstructive pulmonary disease},
  language     = {eng},
  number       = {3},
  pages        = {582--586},
  publisher    = {Eur Respiratory Soc},
  series       = {European Respiratory Journal},
  title        = {Ongoing research in Europe: Alpha One International Registry (AIR) objectives and development.},
  url          = {http://dx.doi.org/10.1183/​09031936.00053606},
  volume       = {29},
  year         = {2007},
}