Protocol for the EARCO Registry: a pan-European observational study in patients with α1-antitrypsin deficiency
Status PubMed-not-MEDLINE Jazyk angličtina Země Anglie, Velká Británie Médium electronic-ecollection
Typ dokumentu časopisecké články
PubMed
32154291
PubMed Central
PMC7049712
DOI
10.1183/23120541.00181-2019
PII: 00181-2019
Knihovny.cz E-zdroje
- Publikační typ
- časopisecké články MeSH
RATIONALE AND OBJECTIVES: Alpha-1 antitrypsin deficiency (AATD) is a genetic condition that leads to an increased risk of emphysema and liver disease. Despite extensive investigation, there remain unanswered questions concerning the natural history, pathophysiology, genetics and the prognosis of the lung disease in association with AATD. The European Alpha-1 Clinical Research Collaboration (EARCO) is designed to bring together researchers from European countries and to create a standardised database for the follow-up of patients with AATD. STUDY DESIGN AND POPULATION: The EARCO Registry is a non-interventional, multicentre, pan-European, longitudinal observational cohort study enrolling patients with AATD. Data will be collected prospectively without interference/modification of patient's management by the study team. The major inclusion criterion is diagnosed severe AATD, defined by an AAT serum level <11 µM (50 mg·dL-1) and/or a proteinase inhibitor genotype ZZ, SZ or compound heterozygotes or homozygotes of other rare deficient variants. Assessments at baseline and during the yearly follow-up visits include lung function testing (spirometry, body plethysmography and diffusing capacity of the lung), exercise capacity, blood tests and questionnaires (symptoms, quality of life and physical activity). To ensure correct data collection, there will be designated investigator staff to document the data in the case report form. All data will be reviewed by the EARCO database manager. SUMMARY: The EARCO Registry aims to understand the natural history and prognosis of AATD better with the goal to create and validate prognostic tools to support medical decision-making.
Alpha 1 UK Support Group Droitwich UK
Clinic for Pneumology DZL Medical University Hannover Hannover Germany
Clinic for Pulmology Clinical Center of Serbia Belgrade Serbia
Dept of Internal Medicine Herlev and Gentofte University Hospital Hellerup Denmark
Dept of Internal Medicine Respiratory Disease Unit Spedali Civili Brescia Italy
Dept of Pulmonary Diseases Celal Bayar University Faculty of Medicine Manisa Turkey
Dept of Pulmonology Leiden University Medical Center Leiden The Netherlands
Dept of Respiratory Diseases Jordanovac University of Zagreb School of Medicine Zagreb Croatia
Dept of Respiratory Medicine and Allergology Skåne University Hospital Lund University Malmö Sweden
Dept of Respiratory Medicine Mater Dei Hospital Msida Malta
Dept of Respiratory Medicine University Hospitals of Coventry and Warwickshire Coventry UK
Division of Pulmonology University Hospital Zurich Zurich Switzerland
Faculty of Medicine University of Belgrade Belgrade Serbia
Faculty of Medicine University of Latvia Riga Latvia
Faculty of Medicine University of Medicine Oradea Bucharest Romania
Hospices Civils de Lyon Service de Pneumologie Hôpital Louis Pradel Lyon France
Institute of Applied Health Research University of Birmingham Birmingham UK
Lung Clinic Tartu University Hospital Tartu Estonia
Marius Nasta Institute Bucharest Romania
Patientenorganisation Alpha1 Deutschland e 5 Gernsheim Germany
Pneumology Dept Hospital Universitari Vall d'Hebron Vall d'Hebron Research Institute Barcelona Spain
Pneumology Dept Tartu University Tartu Estonia
Pulmonary Clinic University Hospital Pleven Bulgaria
Pulmonology Dept Centro Hospitalar de São João Porto Portugal
Respiratory Medicine Cambridge University Hospitals NHS Foundation Trust Cambridge UK
Respiratory Medicine University Hospitals Birmingham NHS Foundation Trust Birmingham UK
Riga East University Hospital Riga Latvia
Université de Lyon Université Lyon 1 UMR754 INRA Lyon France
University Hospitals Leuven Department of Respiratory Diseases Leuven Belgium
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Risk of lung disease in the PI*SS genotype of alpha-1 antitrypsin: an EARCO research project
