A31 MECHANISMS IN CYSTIC FIBROSIS AND OTHER BRONCHIECTATIC DISEASES: Alpha-1 Antitrypsin Is Less Effective At Inhibiting Neutrophil Elastase Activity And Cxcr1 Cleavage In Cystic Fibrosis Versus Non-Cf Bronchiectasis Respiratory Secretions Due To The Actions Of Other Serine And Non-Serine Proteases

Introduction: The major target in treating the proteolytic burden in cystic fibrosis (CF) and non-CF bronchiectasis (NCFB) is neutrophil elastase (NE), which is inhibited by alpha-1 antitrypsin (AAT). Conclusion/Discussion: The clinical implication of these results is that anti-NE therapy alone may be insufficient to address the protease burden in the CF lung, and may require combination with other protease inhibitors.