Update on research, diagnosis and management of alphal-antitrypsin deficiency

Formed in response to a World Health Organization recommendation, the Alpha One International Registry (AIR) is a multinational research program focused on alphal-antitrypsin (AAT) deficiency. Each of the nearly 20 participating countries maintains a National Registry of patients with AAT deficiency and contributes to an international database in Malmö, Sweden, that is designed to increase understanding of AAT deficiency as well as safeguard patient confidentiality. AIR members are engaged in active and wide-ranging investigations to improve the diagnosis, monitoring and therapy of the disease. The AIR membership meets biennially to exchange views and research findings. The third biennial meeting was held in Barcelona, Spain, June 11 -13, 2003. A wide range of AAT deficiency-related topics were addressed, encompassing molecular and cellular pathophysiologic mechanisms, clinical epidemiology, diagnostic advances, current and investigational therapeutic approaches, and progress in registry development. Valuable cross-fertilization of concepts and scientific observations was apparent between AAT deficiency research and other fields of biomedicine. The proceedings of the meeting are summarized in this report.