학술논문

Clinical and functional characteristics of individuals with alpha-1 antitrypsin deficiency: EARCO international registry
Document Type
article
Source
Respiratory Research, Vol 23, Iss 1, Pp 1-12 (2022)
Subject
Registry
Alpha-1 antitrypsin
Phenotypes
Diseases of the respiratory system
RC705-779
Language
English
ISSN
1465-993X
Abstract
Abstract 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 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 registration www.clinicaltrials.gov (ID: NCT04180319)