Burkholderia cepacia in cystic fibrosis. Variable disease course.

Variable clinical course has been reported with the acquisition of Burkholderia cepacia in patients who have cystic fibrosis (CF). We hypothesized that the perceived worsening with B. cepacia may reflect the underlying severity of pulmonary disease at the time of acquisition. To test this hypothesis, we matched CF patients colonized with B. cepacia with CF patients not colonized with the organism. Two-year pre- and postacquisition data and long-term data were compared. Patients were matched for gender, age ( ± 1 yr), height ( ± 5 cm), weight ( ± 8 kg), percent predicted forced expiratory volume in one second (% pred FEV1) ( ± 10%), and pancreatic sufficiency status. Differences in rates of change pre- and postacquisition for FEV1, FVC, weight, and frequency of intravenous courses were compared within pairs with the Wilcoxon signed rank test. Two-year and long-term survival was compared within pairs with the McNemar test. No significant differences were observed in mean annual rates of change in weight (0....