BACKGROUND: Colonisation with @entity6402 is a poor prognostic indicator in subjects with @entity1577 ( @entity1577 ), but outcome prediction is impossible since @entity1 are colonised by different strains with differing pathogenicity. The clinical course of a large cohort of @entity1577 @entity1 colonised with UK epidemic (ET12) @entity6402 was followed for 5 years and compared with that of the remaining @entity1 in the clinic. METHODS: Pulmonary function, nutritional state, and lung pathogen colonisation were recorded for 5 years before December 1997 or @entity204 for all 107 @entity1 who had attended the Liverpool adult @entity1577 clinic since 1993. For each @entity1 a time line from study entry to date of @entity204 or 1997 was constructed. In 1993 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 FEV(1), BMI, and colonisation status across time, and the excess risk associated with @entity6402 colonisation was calculated. Subsequently, in those @entity1 who died between 1993 and 1997, predictive factors for @entity204 were compared within groups using complete 5 year data. RESULTS: Thirty seven @entity1 had been colonised by epidemic @entity6402 and these @entity1 had four times the mortality of the remainder (p<0.01). In 1993 univariate predictors of mortality were age (alive 19.6 (0.64) v dead 23.8 (1.44); p<0.005) and baseline FEV(1) (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 FEV(1) (hazards ratio 1.1, 95% confidence limits 1.06 to 1.14; p<0.001) and colonisation with @entity6402 (hazards ratio 7.92, confidence limits 2.65 to 23.69; p<0.001) were identified as significant factors for @entity204 . Surviving @entity6402 @entity1 had similar initial lung function to the remaining surviving @entity1 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 @entity1 colonised with @entity6402 had better spirometric results than the remaining deceased @entity1 5 years before @entity204 (p<0.05) but lost lung function at a greater rate than non-colonised @entity1 (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 @entity6402 colonisation and shows that those with poor spirometric values are at the greatest risk.

Outcome of @entity6402 colonisation in an adult XXXX centre.

@entity1 :: ('9606', 'Species') :: ['patients', 'patient'] @entity1577 :: ('MESH:D003550', 'Disease') :: ['CF', 'cystic fibrosis'] @entity204 :: ('MESH:D003643', 'Disease') :: ['death'] @entity6402 :: ('292', 'Species') :: ['B cepacia', 'Burkholderia cepacia']
@entity1577 :: (MESH:D003550,Disease) :: ['cystic fibrosis']
