['cystic fibrosis']
['n6022', 'normal saline']

        Inclusion Criteria:

          -  Homozygous for F508del-CFTR gene

          -  Sweat chloride ≥ 60 mEq/L by quantitative pilocarpine iontophoresis

          -  Body weight ≥ 40 kg

          -  FEV1 ≥ 40% predicted

          -  Oxygen saturation ≥ 90% breathing ambient air

          -  Hematology and clinical chemistry of blood and urine results with no clinically
             significant abnormalities that would interfere with the study assessments

          -  Negative pregnancy test for women of child bearing potential

          -  Sexually active subjects of child bearing potential willing to follow contraception
             requirements

        Exclusion Criteria:

          -  Previous enrollment in another cohort for this study.

          -  Any acute infection, including acute upper or lower respiratory infections and
             pulmonary exacerbations that require treatment within 4 weeks of Study Day 1.

          -  Any change in chronic therapies for CF lung disease within 4 weeks of Study Day 1.

          -  Blood hemoglobin <10 g/dL at screening.

          -  Serum albumin <2.5 g/dL at screening.

          -  Abnormal liver function defined as ≥ 3 x upper limit of normal (ULN) in three or more
             of the following: AST, ALT, GGT, ALP, total bilirubin at screening.

          -  History of abnormal renal function (creatinine clearance < 50 mL/min using
             Cockcroft-Gault equation) within a year at screening.

          -  History, including the screening assessment, of ventricular tachycardia or other
             ventricular arrhythmias.

          -  History, including the screening assessment, of prolonged QT and/or QTcF interval (>
             450 msec).

          -  History of solid organ or hematological transplantation.

          -  Intranasal medication changes within 14 days prior to Study Day 1

          -  Required Use of continuous (24 hr/d) or nocturnal supplemental oxygen.

          -  Concomitant use of any inhibitors or inducers of CYP3A4.
      