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CYSTIC FIBROSIS |
1 Manchester Adult Cystic Fibrosis Centre, South Manchester University Hospitals NHS Trust, Wythenshawe Hospital, Manchester M23 9LT, UK
2 Medical Microbiology, University of Edinburgh, Edinburgh EH8 9AG, UK
3 Department of Medical Statistics, South Manchester University Hospitals NHS Trust, Wythenshawe Hospital, Manchester M23 9LT, UK
Correspondence to:
Correspondence to:
Dr A M Jones
Manchester Adult Cystic Fibrosis Centre, South Manchester University Hospitals NHS Trust, Wythenshawe Hospital, Southmoor Road, Manchester M23 9LT, UK; andmarkj{at}hotmail.com
Introduction: Burkholderia cepacia infection has been associated with a poor prognosis for patients with cystic fibrosis (CF). It is now recognised that organisms classified as B cepacia comprise a number of distinct genomic species each known as a genomovar of the B cepacia complex (BCC). The outcome of infection for CF patients with individual genomovars is unknown. The clinical outcome of infection with the two most commonly isolated genomovars (B cenocepacia and B multivorans) was studied at a specialist CF centre between 1982 and 2003.
Methods: The numbers of patients who progressed from initial to chronic infection were assessed. Control groups were created by matching patients with chronic BCC infection by percentage forced expiratory volume in 1 second with patients with Pseudomonas aeruginosa infection. Outcome measures were survival time, deaths from "cepacia syndrome", rate of decline in spirometry and body mass index (BMI), and treatment requirements.
Results: Forty nine patients had an initial infection with either B multivorans (n = 16) or B cenocepacia (n = 33); 8/16 and 31/33, respectively, developed chronic infection (p<0.001). Deaths from "cepacia syndrome" occurred in both BCC groups. Patients with B cenocepacia infection had a shorter survival than patients with P aeruginosa infection (p = 0.01). There was no difference in survival between CF patients infected with B multivorans and P aeruginosa. There were no observed differences in changes in spirometry and BMI or treatment requirements between the BCC groups and respective controls.
Conclusion: In CF, the genomovar status of BCC may influence both the likelihood of progression from initial to chronic infection and the overall survival of the patients.
Keywords: Burkholderia cepacia complex; Burkholderia multivorans; Burkholderia cenocepacia; genomovars; cystic fibrosis
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Thorax 2004 59: 913.
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