Abstract
Introduction: Organisms of the Burkholderia cepacia complex (BCC) are important pathogens in cystic fibrosis (CF). The majority of those who acquire BCC develop chronic infection but it can also result in rapid decline in a significant minority. In addition, chronic infection with Burkholderia cenocepacia in particular is regarded as a contraindication to lung transplantation in many units. Whilst aggressive antibiotic therapy is employed in CF to eradicate Pseudomonas aeruginosa before infection becomes irreversibly established, no formal assessment of such strategies has been previously reported for BCC, despite the apparent widespread adoption of this practice. Methods: UK adult CF centers were surveyed about their current approach to new BCC infection. Outcomes of eradication therapy were assessed in patients attending the Manchester Adult CF Center with new BCC isolates between 1st January 2002 and 1st May 2011. Patients with previous infection with the same strain of BCC were excluded. BCC were identified at the national reference laboratories and confirmed by species-specific PCR and RecA sequencing. Results: Routine use of therapies to attempt eradication of new BCC is commonly used in the UK (12/17 centers who responded). This typically involves a combination of intravenous and nebulised antibiotics. Of 19 eligible cases of new BCC infection, the organism has been eradicated in 7 (37%). Three of these did not receive specific eradication therapy. Of 14 patients who have received eradication therapy and completed follow up, BCC were cleared in only 4 (29%). Conclusions: Attempted eradication of new BCC is a common practice in UK adult CF centers. A minority of patients clear the infection spontaneously and the effects of eradication therapies are at best modest. Early treatment may be associated with better outcomes, though there are insufficient data to support the use of any specific treatment regimen. A prospective, systematic evaluation of treatments and outcomes is required.
Highlights
Organisms of the Burkholderia cepacia complex (BCC) are important pathogens in cystic fibrosis (CF)
Chronic infection tends to be characterized by Staphylococcus aureus but in older subjects is dominated by gram negative organisms, Pseudomonas aeruginosa which infects around 60% of adult CF patients in the UK (CF Trust, 2009)
CURRENT APPROACHES TO NEW BCC INFECTION Responses were obtained from 36 CF units (75% of all UK specialist CF centers), representing over 6300 patients and including 19 pediatric and 17 adult centers
Summary
Organisms of the Burkholderia cepacia complex (BCC) are important pathogens in cystic fibrosis (CF). Outcomes of eradication therapy were assessed in patients attending the Manchester Adult CF Center with new BCC isolates between 1st January 2002 and 1st May 2011. One of the most important groups of organisms causing infection in the CF lung are the those of the Burkholderia cepacia complex (BCC). These currently infect around 3% of UK patients overall, and are predominantly found in adult patients, where the prevalence is four times greater than in those under 16 years (CF Trust, 2009)
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