ABSTRACT
The cystic fibrosis (CF) is typified by a continuous neutrophil-dominated inflammatory response to chronic infection with Pseudomonas aeruginosa. The factors that predispose the CF patient to infection with this particular organism are not clear, but it is increasingly obvious that the inability to eradicate this organism leads to a vicious cycle of neutrophil influx, inflammatory damage, and continued infection. It had long been suspected that the primary defect in CF would be related to an ion transport defect because of the well-recognized, characteristic increase in NaCl concentration of CF sweat that is used as a major diagnostic criterion. Outside of the lung, there is little evidence that inflammation plays a major role in the pathology of CF or that inflammatory processes are regulated abnormally. The link between mutations in the CF gene, its biochemical consequences, and the increased susceptibility to lung infection, particularly with mucoid forms of P. aeruginosa, remains one of the most important unanswered questions in CF research.
