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

¹ Dept of Medicine, Queen Elizabeth Hospital, Edgbaston, Birmingham, and ⁵ Pharmanet, Buckingham Court, Kingsmead Business Park, High Wycombe, UK. ² Clinica Malattie Apparto Respiraorio, IRCCS Policlinico San Mateo, Università di Pavia, Pavia, Italy. ³ Servei de Pneumologia, Institut Clínic del Tòrax (IDIBAPS), Hospital Clínic, Barcelona, Spain. ⁴ Dept of Respiratory Medicine, University Hospital, Malmö, Sweden.

CORRESPONDENCE: R. A. Stockley, Dept of Medicine, University Hospital Birmingham, Edgbaston, Birmingham, B15 2TH, UK. Fax: 44 1216978257. E-mail: r.a.stockley{at}bham.ac.uk

Keywords: Augmentation therapy, chronic obstructive pulmonary disease, emphysema, epidemiology, prevalence, registries

Received: April 19, 2006
Accepted October 22, 2006

In 1997, the World Health Organization recommended establishing an international registry of 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, Malmö, 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 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 ₁-antitrypsin deficiency from various ethnic groups.

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