Augmentation Therapy for Severe Alpha-1 Antitrypsin Deficiency Improves Survival and Is Decoupled from Spirometric Decline—A Multinational Registry Analysis

Author:

Fraughen Daniel D.1ORCID,Ghosh Auyon J.2ORCID,Hobbs Brian D.3ORCID,Funk Georg-Christian4ORCID,Meischl Tobias45ORCID,Clarenbach Christian F.6ORCID,Sievi Noriane A.6ORCID,Schmid-Scherzer Karin4,McElvaney Oliver J.17,Murphy Mark P.1ORCID,Roche Adam D.1ORCID,Clarke Louise8,Strand Matthew9ORCID,Vafai-Tabrizi Florian4ORCID,Kelly Geraldine1ORCID,Gunaratnam Cedric8ORCID,Carroll Tomás P.1ORCID,McElvaney Noel G.1ORCID

Affiliation:

1. Department of Medicine, Irish Center for Genetic Lung Disease, Royal College of Surgeons in Ireland, Dublin, Ireland;

2. Division of Pulmonary, Critical Care, and Sleep Medicine, SUNY Upstate University Hospital, Syracuse, New York;

3. Channing Division of Network Medicine and Division of Pulmonary and Critical Care Medicine, Brigham and Women’s Hospital, Boston, Massachusetts;

4. Karl Landsteiner Institute for Lung Research and Pulmonary Oncology and Department of Medicine II with Pneumology, Klinik Ottakring, Vienna, Austria;

5. Division of Gastroenterology and Hepatology, Department of Medicine III, Medical University of Vienna, Vienna, Austria;

6. Department of Pneumology, University Hospital Zurich, Zurich, Switzerland;

7. Division of Pulmonary, Critical Care and Sleep Medicine, University of Washington, Seattle, Washington;

8. Department of Respiratory Physiology, Beaumont Hospital, Dublin, Ireland; and

9. Division of Biostatistics and Bioinformatics, National Jewish Health, Denver, Colorado

Funder

Alpha-1 Foundation

Alpha-1 Antitrypsin Laurell’s Training Award

Charitable Infirmary Charitable Trust

Health Research Charities Ireland

Publisher

American Thoracic Society

Subject

Critical Care and Intensive Care Medicine,Pulmonary and Respiratory Medicine

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