Cystic fibrosis lung disease is characterized by chronic bacterial infection and inflammation dominated by neutrophils resulting in excessive release of neutrophil elastase. Endogenous elastase inhibitors are not adequate to neutralize neutrophil elastase. Aerosol administration of Prolastin, an elastase inhibitor, to cystic fibrosis patients will be undertaken with the goal of determining the dose needed to neutralize the excess elastase activity.
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