title: Effect of Burkholderia cepacia infection in the clinical course of patients with cystic fibrosis: a pilot study in a Sydney clinic.
abstract: BACKGROUND
  Colonisation with Burkholderia cepacia complex in patients with cystic fibrosis (CF) has been associated with adverse outcomes. The aim of the present study was to determine the actuarial survival of CF patients colonized with B. cepacia and to evaluate the efficacy of the Royal Prince Alfred Hospital segregation policy. A secondary aim was to characterize the specific genomovars and strains of B. cepacia isolated in an Australian clinic.
  
  
  METHODS
  Retrospective review of spirometric and microbiological data on all patients colonized with B. cepacia. Each B. cepacia-colonized subject was matched with three case-control subjects. Phenotype and genomovar typing, random amplified polymorphic DNA strain type and B. cepacia epidemic strain marker analyses were performed. The effect of B. cepacia colonization on transplant-free survival was estimated by Cox's proportional hazards regression using the entire clinic population.
  
  
  RESULTS
  Fifteen patients were colonized with B. cepacia, of whom six (40%) had died from CF-related disease by August 1998, compared with 30 of 173 (17.3%) of the entire clinic population. Cepacia status had a significant adverse effect on survival, with a hazard ratio of 2.16 (95% confidence interval 1.0-4.69; P = 0.05). The outcome was variable in subgroups of B. cepacia.
  
  
  CONCLUSIONS
  Colonization with B. cepacia had a significant adverse effect on survival within the study population. Genomovar and strain typing contributed usefully in accessing the effectiveness of the hospital's segregation policy in preventing cross-colonization.
authors.affiliation: Department of Respiratory Medicine, Gosford Hospital, Gosford, New South Wales, Australia. rsoni@mail.usyd.edu.au
authors.forename: Rajeev
authors.initials: R
authors.lastname: Soni
authors.forename: Guy
authors.initials: G
authors.lastname: Marks
authors.forename: Deborah A
authors.initials: DA
authors.lastname: Henry
authors.forename: Michael
authors.initials: M
authors.lastname: Robinson
authors.forename: Carmel
authors.initials: C
authors.lastname: Moriarty
authors.forename: Stephen
authors.initials: S
authors.lastname: Parsons
authors.forename: Peter
authors.initials: P
authors.lastname: Taylor
authors.forename: Eshwar
authors.initials: E
authors.lastname: Mahenthiralingam
authors.forename: David P
authors.initials: DP
authors.lastname: Speert
authors.forename: Peter T
authors.initials: PT
authors.lastname: Bye
mesh_terms: D000206:Actuarial Analysis; D000293:Adolescent; D000328:Adult; D015373:Bacterial Typing Techniques; D019121:Burkholderia Infections / Q000150:complications*; D016956:Burkholderia cepacia / Q000145:classification; D003430:Cross-Sectional Studies; D003550:Cystic Fibrosis / Q000382:microbiology* / Q000401:mortality*; D006801:Humans; D008875:Middle Aged; D009517:New South Wales / Q000453:epidemiology; D010865:Pilot Projects; D016016:Proportional Hazards Models; D015656:Respiratory Mechanics; D012189:Retrospective Studies