Safety of AZLI in Children With Cystic Fibrosis (CF) and Chronic Pseudomonas Aeruginosa in the Lower Airways

This is an open-label, multicenter study in children ≤ 12 years of age with CF and chronic
PA infection in the lower airways. This study will be conducted at approximately 30 centers
in Europe and the United States. The total study duration will be approximately 7 months.

Eligible subjects will receive study treatment as 3 intermittent 28-day courses of AZLI 75
mg TID administered via the Investigational eFlow Nebulizer System, each followed by 28 days
off AZLI.

Inclusion Criteria:

- Males or females 12 years of age or younger.

- Diagnosis of CF as determined by the 1997 CF Consensus Conference criteria:

- Documented sweat chloride ≥ 60 mEq/L by quantitative pilocarpine iontophoresis
test OR

- Abnormal nasal transepithelial potential difference (NPD) test OR

- A genotype with 2 identifiable mutations consistent with CF AND

- One or more clinical features consistent with CF.

- Documented positive lower respiratory tract culture for PA at the screening visit
plus two documented positive lower respiratory tract cultures for PA within 12 months
prior to study entry (must be a minimum 3 months apart.)

- Clinically stable with no evidence of significant respiratory symptoms or, if
obtained for clinical evaluation, no chest radiograph findings at screening that
would require administration of IV antipseudomonal antibiotics, oxygen
supplementation, or hospitalization.

Exclusion Criteria:

- Use of IV or inhaled antipseudomonal antibiotics within 14 days of study entry

- Presence of a condition or abnormality that would compromise the subject's safety or
the quality of study data, in the opinion of the investigator

- History of sputum or throat swab culture yielding Burkholderia spp. within 2 years
prior to Screening Visit

- History of hypersensitivity/adverse reaction to aztreonam

- History of hypersensitivity/adverse reaction to beta-agonists

- History of lung transplantation

- Administration of any investigational drug or device within 30 days prior to
Screening Visit or within 6 half-lives of the investigational drug (whichever is
longer)

- Hospitalization for pulmonary-related illness within 28 days prior to Screening Visit

- Changes in or initiation of chronic azithromycin treatment within 28 days prior to
Screening Visit

- Changes in or initiation of hypertonic saline treatment within 7 days prior to
Screening Visit; for subjects on a stable regimen of hypertonic saline (28 days on/28
days off), beginning or ending a cycle of hypertonic saline is allowed

- Changes in antimicrobial, bronchodilator (BD), corticosteroid or dornase alfa
medications within 7 days prior to Screening Visit;

- Changes in physiotherapy technique or schedule within 7 days prior to Screening Visit

- Abnormal renal or hepatic function results at most recent test within the previous 90
days.