Abstract

The Burkholderia cepacia complex is comprised of a group of related bacterial species that are capable of causing life-threatening respiratory tract infection in persons with cystic fibrosis. This article reviews advances in our understanding of Burkholderia cepacia complex infection in cystic fibrosis, focusing on the taxonomy, clinical microbiology, and epidemiology, as well as the natural history and clinical outcomes associated with Burkholderia cepacia complex infection. Each of the nine species of the Burkholderia cepacia complex has now received a formal species name. These names are the preferred nomenclature, replacing the former 'genomovar' designations. Studies from several countries reiterate that two species, Burkholderia cenocepacia and Burkholderia multivorans, account for most Burkholderia cepacia complex infection in cystic fibrosis. Bacterial genotyping studies indicate that specific Burkholderia cepacia complex strains infect multiple cystic fibrosis patients, implying that they may have an enhanced capacity for interpatient spread. Emerging clinical outcomes data suggest that at least some of these so-called transmissible or epidemic strains are also more virulent in the cystic fibrosis host. Ongoing research is aimed at gaining a better understanding of Burkholderia cepacia complex ecology, defining Burkholderia cepacia complex virulence factors and pathogenic mechanisms, and determining the relative virulence of distinct strains. Significant advances in our understanding of the Burkholderia cepacia complex serve as a critical foundation for further efforts that ultimately will enable better infection control and the development of novel therapeutics to treat Burkholderia cepacia complex infection in persons with cystic fibrosis.

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