ABSTRACT
Cystic fibrosis (CF) is the most common life-shortening autosomal recessive disorder in north-west European populations.1, 2 The disease is a consequence of mutations of the cystic fibrosis transmembrane regulator (CFTR) gene, which encodes for a complex chloride ion channel expressed on the apical surface of epithelial cells.3-5 The primary organs affected are the lungs, pancreas, and liver. Over 1300 mutations of this gene have been described and the prevalence of individual mutations varies between countries.2, 6, 7
Most people with cystic fibrosis die from respiratory failure.8 This is a consequence of progressive lung damage resulting from chronic infection. A host bacterial relationship is established with bronchial epithelial and alveolar tissues becoming injured owing to the effects of bacterial proteases, host defense proteases, and oxygen derived free radicals.9, 10 The relationship between infection and abnormal function of the cystic fibrosis transmembrane regulator (CFTR) protein has become much clearer in the past 5 years.11 A number of hypotheses have been advanced to explain the susceptibility to infection but increasingly the evidence indicates that CFTR dysfunction causes dehydration of periciliary fluid.12 Cilia cannot function effectively with reduced volume of airway surface liquid (ASL) and mucociliary clearance is impaired.13 This impairment of innate defense promotes early infection and inflammation as antigenic inhaled particles and bacteria are not efficiently cleared.14
