DMP 777 LUNG SPECIFIC ORAL ADMINISTRATION IN PATIENTS WITH CYSTIC FIBROSIS

主要目的是评估口服DMP 777 14天后，囊性纤维化(CF)成人患者支气管肺泡灌洗液(BAL)中人中性粒细胞弹性蛋白酶(NE)细胞外抑制活性的大小，并将这种影响与观察到的BAL液中细胞外HNE活性的变化与系统药代动力学(PK)和药效(PD)数据相关联。评估DMP 777的安全性。第二个目标是确定BAL细胞外HNE抑制的程度与与CF肺部疾病发病机制相关的生物学指标改善之间的相关性。

The primary objectives are to assess the magnitude of extacellular human neutrophil elastase (NE) ingibitory activity in bronchoalveolar lavage (BAL) fluid following 14 days of oral DMP 777 administration twice daily or three times daily in adult patients with cystic fibrosis (CF) and to correlate this effect with any observed changes in extracellualar HNE activity in BAL fluid with systmeic pharmacokinetic (PK) and pharmacodynamic (PD) data. Evaluate the safety of DMP 777. The secondary objective is to determine the correlation between the magnitude of BAL extracellular HNE inhibition and improvement in biological indices relevant to the pathogenesis of lung disease in CF.