Pseudomonas acquisition in young patients with cystic fibrosis: pathophysiology, diagnosis, and management

Abstract

Purpose of review: To summarize the pathophysiology of, risk factors for, and outcomes of early Pseudomonas aeruginosa (Pa) infection in CF; to review the results of trials of early intervention and to describe treatment options for early Pa infection.

Recent findings: Chronic lower airway Pa infection is associated with significant morbidity and mortality among CF patients. However, first acquisition of Pa does not appear to cause an immediate and rapid decline in lung function. Early Pa isolates are generally non-mucoid, antibiotic-sensitive, and present at low density, suggesting a possible "window of opportunity" for early intervention.

Summary: Anti-pseudomonal therapy for early infection results in transient Pa eradication, but re-infection with Pa appears inevitable despite early aggressive treatment. There are no controlled trials demonstrating clinical benefit in young children. There is a critical need for further investigation of the clinical outcomes associated with early intervention, the long-term safety profile, and the optimal drug regimen.