Burkholderia cepacia complex in cystic fibrosis: critical gaps in diagnosis and therapy

Abstract

Burkholderia cepacia complex (Bcc) is a bacterial group with ‘natural’ multi-antimicrobial resistance. This complex has generated epidemic outbreaks across the world. In people with cystic fibrosis (CF), Bcc can cause severe lung infections that lead to accelerated lung damage, which can be complicated by necrotizing pneumonia accompanied by high fevers, leucocytosis, and bacteraemia, which commonly causes fatal outcomes. Specifically, infection by Burkholderia cenocepacia is considered an exclusion criterion for lung transplantation. The species of Bcc exhibit both genetic and phenotypic hypervariability that complicate their accurate microbiological identification. Automated methods such as MALDI-TOF can err in the determination of species. Their slow growth even in selective agars and the absence of international consensuses on the optimal conditions for their isolation make early diagnosis a difficult challenge to overcome. The absence of correlations between antibiograms and clinical results has resulted in the absence of standardized cut-off values of antimicrobial susceptibility, a fact that brings a latent risk since incorrect antibiotic therapy can induce the selection of more aggressive variants that worsen the clinical picture of the host, added to the absence of a clear therapeutic guide for the eradication of pulmonary infections by Bcc in patients with CF, resulting in frequently ineffective treatments. There is an urgent need to standardize methods and diagnostic tools that would allow an early and accurate diagnosis, as well as to perform clinical studies of the effectiveness of available antibiotics to eradicate Bcc infections, which would allow us to establish standardized therapeutic schemes for Bcc-infected patients.

Keywords:

• Burkholderia cepacia complex
• cystic fibrosis
• antimicrobial stewardship
• early diagnosis
• antibacterial agents

Authors contributions

Both authors contributed to the creation of the review article as follows: Conceptualization: Juan Carlos Gutiérrez Santana; Resources: Victor Rafael Coria-Jiménez; Supervision: Victor Rafael Coria-Jiménez: Writing – original draft: Juan Carlos Gutiérrez-Santana; Writing – review & editing: Victor Rafael Coria-Jiménez. Both authors have reviewed the manuscript and vouch for the accuracy and completeness of the data.

Disclosure statement

The authors declare the absence of conflicts of interest.

Data availability statement

The data presented in this review were taken from published literature on public domains and therefore data sharing is not applicable to this article, as no new data were created or analysed in this study.

Additional information

Funding

Juan Carlos Gutiérrez Santana benefited from the support of scholarship 2018-000068-02NACF-28106 from the National Council of Science and Technology (CONACyT), Mexico, thanking this organization for its support. This work is funded by federal funds, Mexico, from the National Institute of Paediatrics, authorization 068/2019.