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. 2016 Jan 20;2016(1):CD009529.
doi: 10.1002/14651858.CD009529.pub3.

Antibiotic Treatment for Burkholderia Cepacia Complex in People With Cystic Fibrosis Experiencing a Pulmonary Exacerbation

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Antibiotic Treatment for Burkholderia Cepacia Complex in People With Cystic Fibrosis Experiencing a Pulmonary Exacerbation

Alex Horsley et al. Cochrane Database Syst Rev. .
Free PMC article

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Background: Chronic pulmonary infection is a hallmark of lung disease in cystic fibrosis. Infections dominated by organisms of the Burkholderia cepacia complex, a group of at least 18 closely-related species of gram-negative bacteria, are particularly difficult to treat. These infections may be associated with a fulminant necrotising pneumonia. Burkholderia cepacia complex bacteria are resistant to many common antibiotics and able to acquire resistance against many more. Following patient segregation in cystic fibrosis medical care, the more virulent epidemic strains are not as frequent, and new infections are more likely to be with less virulent environmentally-acquired strains. Although evidence-based guidelines exist for treating respiratory exacerbations involving Pseudomonas aeruginosa, these cannot be extended to Burkholderia cepacia complex infections. This review, which is an update of a previous review, aims to assess the available trial evidence for the choice and application of treatments for these infections.

Objectives: To assess the effectiveness and safety of different antibiotic regimens in people with cystic fibrosis experiencing an exacerbation and chronically infected with organisms of the Burkholderia cepacia complex.

Search methods: We searched the Cochrane Cystic Fibrosis Trials Register, compiled from electronic database searches and handsearching of journals and conference abstract books. We also searched the reference lists of relevant articles and reviews.Date of latest search: 28 August 2015.

Selection criteria: Randomised and quasi-randomised controlled trials of treatments for exacerbations of pulmonary symptoms in people with cystic fibrosis chronically infected with organisms of the Burkholderia cepacia complex.

Data collection and analysis: No relevant trials were identified.

Main results: No trials were included in this review.

Authors' conclusions: Burkholderia cepacia complex infections present a significant challenge for people with cystic fibrosis and their clinicians. The incidence is likely to increase as the cystic fibrosis population ages; and managing and treating these infections will become more important. There is a lack of trial evidence to guide decision making and no conclusions can be drawn from this review about the optimal antibiotic regimens for people with cystic fibrosis who have chronic Burkholderia cepacia complex infections. Clinicians must continue to assess each person individually, taking into account in vitro antibiotic susceptibility data, previous clinical responses and their own experience. Multicentre randomised clinical trials are needed to assess the effectiveness of different antibiotic regimens in people with cystic fibrosis infected with organisms of the Burkholderia cepacia complex.

Conflict of interest statement

AH declares that his institution has received money from Celtaxys Pharmaceuticals (consultancy), Vertex Pharamceuticals (grant to host a one day CF conference in Manchester in 2016) and Markedsmodingsfonden (grant from Danish Government to assist in improving the clinical utility of the Innocor multiple breath washout system; funds supported research nurse to use the system and feedback on how to improve usability). AH has applied to the UK CF Trust as part of two Strategic Research Consortium bids (money payable to his institution) both of which are multi‐centre collaborative efforts to address different areas of CF care, unrelated to the current review. He has personally received an honorarium from Gilead Pharmaceuticals for presenting at the Expert Viewpoints in CF meeting, 2014 and received royalties from Oxford University Press from the sale of Oxford Respiratory Medicine Library Handbook on Cystic Fibrosis, published March 2015 (2nd edition).

AJ declares the receipt of funds for advisory board work for Gilead Pharmaceuticals. His institution has received contributions from Forest Pharmaceuticals and Gilead Pharmaceuticals for AH to travel to conferences. He has participated in a multicentre project evaluating clinical outcome of use of nebulised aztreonam (sponsored by Gilead Sciences) and in January 2015 he spoke at the national Irish CF Meeting (meeting and AJ travel to there sponsored by Gilead). AJ further declares sponsorship from Forest Pharmaceuticals for the CF Centre’s annual away day team meeting and an annual meeting with regional paediatric teams (which he helps organise and run). Vertex Pharmaceuticals provided an unrestricted educational grant to his institution to fund a one‐day CF meeting in Manchester in 2016.

RL declares no known conflict of interest.


Study flow diagram. Please note: some studies had more than one reason for exclusion. All are included here, although only the major reason is described in the text.

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