SUMMARY OF A WORKSHOP ON INFECTIONS IN PATIENTS WITH CYSTIC-FIBROSIS

Cystic fibrosis (CF) is currently the most common fatal hereditary disease of the Caucasian race. The incidence of this disease is approximately one in every 2, 000 live births, and the mean age of survival is 20 years. The leading cause of death is respiratory failure, usually after a long history of purulent bronchitis. Pulmonary infections in CF are associated with a distinctive and predictable bacterial etiology. Before antibiotics were available, Staphylococcus aureus was the organism most frequently isolated from the sputum of a CF patient. With the advent of effective antistaphylococ-cal drugs, the predominant infectious agent in the CF lung has become Pseudomonas aeruginosa. Of considerable interest is the observation that the majority of Pseudomonas strains isolated from chronically infected CF patients produce a peculiar mucoid slime substance. This characteristic has rarely been noted for pathogenic Pseudomonas isolates from non-CF patients. It is also apparent that the reason for persistent pseudomonas infection in CF patients is not an inability to produce circulating antibodies to Pseudomonas. © 1979 by The University of Chicago.