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Background: Colonisation with Burkholderia cepacia is a poor prognostic indicator in subjects with cystic fibrosis (CF), but outcome prediction is impossible since patients are colonised by different strains with differing pathogenicity. The clinical course of a Jorge cohort of CF patients colonised with UK epidemic (ET1 2) B cepacia was followed for 5 years and compared with that of the remaining patients in the clinic. Methods: Pulmonary function, nutritional state, and Jung pathogen colonisation were recorded for 5 years before December 1997 or death for all 107 patients who had attended the Liverpool adult CF clinic since 1993. For each patient a time line from study entry to date of death or 1997 was constructed. In 1 993 potential risk factors including age and sex were subjected to Cox proportional hazards analysis using the end point of mortality as the outcome variable. The analysis was supplemented by time varying covariables that described the change in FEV1, BMI, and colonisation status across time, and the excess risk associated with B cepacia colonisation was calculated. Subsequently, in those patients who died between 1993 and 1 997, predictive factors for death were com- pared within groups using complete 5 year data. Results: Thirty seven patients had been colonised by epidemic B cepacia and these patients had four times the mortality of the remainder (p<0.0l). In 1993 univariate predictors of mortality were age (alive 19.6 (0.64) vdead 23.8 (1.44); p<0.005) and baseline FEV1 (alive 68.6 (2.5)% predicted v dead 43.2 (4.8)%; p<0.001) with a trend for BMI (p=0.07). However, following time varying covariate Cox proportional hazards analysis, only lower FEV1 (hazards ratio 1.1, 95% confidence limits 1 .06 to 1.14; p<0.001) and colonisation with B cepacia (hazards ratio 7.92, confidence limits 2.65 to 23.69; p<0.001) were identified as significant factors for death. Surviving B cepacia patients had similar initial lung function to the remaining surviving patients but had an accelerated loss of lung function over the study period (colonised -1.9% predicted per year v non-colonised -0.3% predicted per year; p<0.05). Deceased patients colonised with B cepacia had better spirometric results than the remaining deceased patients 5 years before death (p<0.05) but lost lung function at a greater rate than non-colonised patients (colonised -6.2% predicted per year v non-colonised -1 .9% predicted per year; p<0.05). Conclusions: This study confirms the excess mortality associated with epidemic B cepacia colonisation and shows that those with poor spirometric values are at the greatest risk. [ABSTRACT FROM AUTHOR]

Although dornase alpha (recombinant human DNase) can thin the viscid pulmonary secretions of cystic fibrosis (CF), clinical trials in groups of unselected patients have shown only modest average improvements in pulmonary function. The product is very expensive, so in conjunction with purchasers we designed selection criteria and a protocol for a 2-week trial to target CF individuals who might gain most benefit. Treatment was to be continued in those showing ⩾10% improvement in pulmonary function. Those who had a trial of dornase alpha were followed up for 2 years. Of 25 patients who had a 2-week trial of dornase alpha, 17 met the criteria for continuation (average gain in forced expiratory volume 37%). The 11 of these who were still alive at 2 years had a greater initial average FEV1 improvement than those who had died (45% versus 22%), and still had an average improvement of 31% at 2 years. The 8 patients who did not meet the criteria for continuation were older and had required fewer intravenous antibiotic courses. All these were alive at 2 years with unchanged clinical indices. This method of selection for dornase alpha treatment allows targeting to those who gain most benefit without disadvantaging the remaining patients. Furthermore, production of such guidelines in conjunction with purchasers obviates funding difficulties and allows rational prescribing. [ABSTRACT FROM AUTHOR]

Colonisation with Pseudomonas aeruginosa is common in adults with cystic fibrosis (CF) and there is increasing evidence that transmissible strains may cross colonise patients. However, transmission of these strains by social contact to healthy non-CF individuals has not been described. A case is presented where an adult CF patient colonised by an epidemic P aeruginosa strain infected her parents with subsequent morbidity. [ABSTRACT FROM AUTHOR]