Abstract
Cystic fibrosis (CF), a condition produced by mutations in the gene that encodes the cystic fibrosis transmembrane conductance regulator, is the most prevalent autosomal-recessive hereditary disease in caucasian populations. Among other repercussions, this defect leads to an alteration of respiratory secretions and determines a predisposition for chronic bronchopulmonary colonization-infection, which is the main driver of the high morbidity and early mortality of CF patients. Colonization by Staphylococcus aureus and Haemophilus influenzae is frequent in children younger than 10 years, but mucoid Pseudomonas aeruginosa is by far the most relevant pathogen in adults with CF and is responsible for the progressive bronchopulmonary deterioration. As a consequence of repeated, long-lasting antimicrobial treatments and deterioration of lung function, colonization by multidrug-resistant Gram-negative bacilli, such as Stenotrophomonas maltophilia, Achromobacter spp. and Burkholderia cepacia complex, is also frequent in adult CF patients. The special characteristics of the pathologic process and the microorganisms implicated in CF make it advisable to consider microbiological follow-up of chronic bronchopulmonary colonization-infection in these patients a specific diagnostic entity.