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Multidrug-Resistant Pseudomonas aeruginosa in Cystic Fibrosis.Part 1: Epidemiology, Mechanisms, and Clinical Implications

Michael D Parkins1,2, Christopher D Sibley1, and J Stuart Elborn3,4

Cystic fibrosis (CF) lung disease is characterized by thickened secretions that impair mucociliary clearance, thereby reducing the normal removal of inhaled bacteria and other particulates. This environment in the airways promotes bacterial and host factors that favor the development of chronic lower airway infection [1]. As the disease progresses, repeated episodes of infection and inflammation lead to lung injury manifesting as bronchiectasis and, ultimately, end-stage respiratory disease. Ultimately, 60–80% of patients with CF become chronically infected with Pseudomonas aeruginosa, the archetypal CF pathogen [2,3]. Chronic infection with P aeruginosa and its conversion to an alginate hyperproducing, mucoid phenotype is associated with progressive decline in lung function, increased risk of hospitalization, and reduced survival [4–8]. Despite the progress that has been made in controlling CF airway disease, death secondary to respiratory failure remains the most common contributor to the early demise of patients.

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