Safety and Efficacy of 2 Treatment Regimens of Aztreonam for Inhalation Solution in Children With Cystic Fibrosis and New Onset Pseudomonas Aeruginosa Infection

Key Inclusion Criteria:

- Diagnosis of Cystic Fibrosis (CF) as determined by the 2008 CF Consensus Conference
criteria: Sweat chloride level ≥ 60 milliequivalents per liter (mEq/L) by quantitative
pilocarpine iontophoresis; or a genotype with 2 identifiable mutations consistent with
CF; or an abnormal nasal transepithelial potential difference (NPD), and 1 or more
clinical features consistent with CF

- Documented new onset of positive respiratory tract culture for PA within 30 days of
screening defined as either first lifetime documented PA-positive culture, or PA
recovered after at least a 2-year history of PA-negative respiratory cultures (at
least 2 cultures per year)

- Forced expiratory volume in one second (FEV1) ≥ 80% predicted (for subjects ≥ 6 years
of age who can reliably perform spirometry assessments)

- Clinically stable with no evidence of acute significant respiratory symptoms that
would require administration of IV antipseudomonal antibiotics, oxygen
supplementation, or hospitalization

Key Exclusion Criteria:

- Use of IV or inhaled antipseudomonal antibiotics within 2 years of screening

- Use of oral antipseudomonal antibiotics for a respiratory event within 30 days of
study entry (screening visit)

- History of intolerance to inhaled short acting β2 agonists

- History of lung transplantation

- Current requirement for daily continuous oxygen supplementation or requirement of more
than 2 L/minute at night

- Hospitalization for a respiratory event within 30 days prior to screening

- Changes in bronchodilator, corticosteroid, dornase alfa, or hypertonic saline
medications within 7 days prior to screening.

- Significant changes (per investigators discretion) in physiotherapy technique or
schedule within 7 days prior to screening

- Abnormal renal or hepatic function results at most recent test within the previous 12
months, defined as Aspartate aminotransferase (AST) or alanine aminotransferase (ALT)
> 5 times upper limit of normal (ULN), or Serum creatinine > 2 times ULN for age

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

- Known hypersensitivity to aztreonam, its metabolites, or formulation excipients in
AZLI

- Respiratory cultures performed within 24 months prior to screening that are positive
for ANY Burkholderia spp. or Non-tuberculous Mycobacteria (NTM)

Note: Other protocol defined Inclusion/Exclusion criteria may apply