Abstract

BackgroundSeveral cases of Burkholderia pseudomallei infection in CF have been previously reported. We aimed to identify all cases globally, risk factors for acquisition, clinical consequences, and optimal treatment strategies.MethodsWe performed a literature search to identify all published cases of B. pseudomallei infection in CF. In addition we hand-searched respiratory journals, and contacted experts in infectious diseases and CF around the world. Supervising clinicians for identified cases were contacted and contemporaneous clinical data was requested.Results25 culture-confirmed cases were identified. The median age at acquisition was 21 years, mean FEV1 % predicted was 60 %, and mean BMI was 19.5 kg/m2. The location of acquisition was northern Australia or south-east Asia for most. 19 patients (76 %) developed chronic infection, which was usually associated with clinical decline. Successful eradication strategies included a minimum of two weeks of intravenous ceftazidime, followed by a consolidation phase with trimethoprim/sulfamethoxazole, and this resulted in a higher chance of success when instituted early. Three cases of lung transplantation have been recorded in the setting of chronic B. pseudomallei infection.ConclusionChronic carriage of B. pseudomallei in patients with CF appears common after infection, in contrast to the non-CF population. This is often associated with an accelerated clinical decline. Lung transplantation has been performed in select cases of chronic B. pseudomallei infection.Electronic supplementary materialThe online version of this article (doi:10.1186/s12890-015-0109-9) contains supplementary material, which is available to authorized users.

Highlights

  • Several cases of Burkholderia pseudomallei infection in cystic fibrosis (CF) have been previously reported

  • Burkholderia pseudomallei is a gram-negative environmental bacterium found in soil and surface water that causes melioidosis, which most commonly occurs in south-east Asia and northern Australia [1]

  • The international experience with B. pseudomallei described here demonstrates that this organism has the potential to exhibit novel behaviours in the CF host, including the development of chronic infection

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Summary

Introduction

Several cases of Burkholderia pseudomallei infection in CF have been previously reported. We aimed to identify all cases globally, risk factors for acquisition, clinical consequences, and optimal treatment strategies. Burkholderia pseudomallei is a gram-negative environmental bacterium found in soil and surface water that causes melioidosis, which most commonly occurs in south-east Asia and northern Australia [1]. Melioidosis has been increasingly recognised to occur in diverse tropical locations globally, including the Americas and Africa [2]. In the 25-year Darwin Prospective Melioidosis Study [6], only one of over 750 consecutive melioidosis patients had evidence of long-term persisting infection with B. pseudomallei.

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