title: [National cohort of patients with emphysema and alpha-1 antitrypsin deficiency].
abstract: BACKGROUND
  The natural history and optimal therapeutic management of emphysema associated with alpha-1 antitrypsin deficiency are poorly understood. The purpose of this cohort of patients with emphysema associated with alpha-1 antitrypsin deficiency is to describe their characteristics and to study the factors associated with the evolution of their pathology.
  
  
  METHODS
  It is a multicentre, prospective, cohort study including all the patients in metropolitan France with alpha-1 antitrypsin deficiency associated with emphysema (diagnosed by CT scanning) and an obstructive ventilatory defect. All respiratory physicians will be approached. Patients meeting the inclusion criteria will be followed up every 6 months for 5 years. Clinical and spirometric data as well as those relating to quality of life will becollected. The principal criterion will be FEV1; the secondary criteria: quality of life, exacerbations requiring hospitalistion and mortality.
  300-500 patients are expected to be included. At the conclusion incorporation of this cohort into AIR (Alpha-antitrypsin International Registry) will increase the statistical power. This study could also provide the base for prospective trials and research projects.
authors.affiliation: Service de Pneumologie et Réanimation Respiratoire, Hôpital Beaujon, Clichy et Département d'Epidémiologie, de Biostatistiques et de Recherche Clinique, Hôpital Bichat, Paris, France. g.thabut@bch.ap-hop-paris.fr
authors.forename: G
authors.initials: G
authors.lastname: Thabut
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D015331:Cohort Studies; D003627:Data Interpretation, Statistical; D005500:Follow-Up Studies; D005602:France; D006760:Hospitalization; D006801:Humans; D008875:Middle Aged; D018579:Patient Selection; D011379:Prognosis; D011446:Prospective Studies; D029424:Pulmonary Disease, Chronic Obstructive / Q000150:complications / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000401:mortality; D011656:Pulmonary Emphysema / Q000150:complications / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000401:mortality; D011788:Quality of Life; D013902:Radiography, Thoracic; D012042:Registries; D013147:Spirometry; D011795:Surveys and Questionnaires; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D019896:alpha 1-Antitrypsin Deficiency / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy / Q000401:mortality