Article
Augmentation Therapy for Severe Alpha-1 Antitrypsin Deficiency Improves Survival and Is Decoupled from Spirometric Decline-A Multinational Registry Analysis.
American journal of respiratory and critical care medicine - 1 Nov 2023
Fraughen Daniel D, Ghosh Auyon J, Hobbs Brian D, Funk Georg-Christian, Meischl Tobias, Clarenbach Christian F, Sievi Noriane A, Schmid-Scherzer Karin, McElvaney Oliver J, Murphy Mark P, Roche Adam D, Clarke Louise, Strand Matthew, Vafai-Tabrizi Florian, Kelly Geraldine, Gunaratnam Cedric, Carroll Tomás P, McElvaney Noel G
Abstract excerpt
Rationale: Intravenous plasma-purified alpha-1 antitrypsin (IV-AAT) has been used as therapy for alpha-1 antitrypsin deficiency (AATD) since 1987. Previous trials (RAPID and RAPID-OLE) demonstrated efficacy in preserving computed tomography of lung density but no effect on FEV1. This observational study evaluated 615 people with severe AATD from three countries with socialized health care (Ireland, Switzerland,...
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