ePS06.9 Lung disease progression and inflammatory parameters in children with cystic fibrosis chronically colonized with Burkholderia cepacia complex

Objectives The aim of this study was to compare lung disease severity and inflammatory markers in cystic fibrosis (CF) pediatric patients chronically colonized with Burkholderia cepacia complex (Bcc) and those who were free of the pathogen. Methods 95 children (mean age 11.5 years) were enrolled into the study. Besides routine examination and pulmonary function tests (PFT) performing, sputum and plasma concentrations of cytokines (TNF-α, IFNγ, IL-4, IL-10, IL-17, TGF-b1) and in situ elastase levels were assayed with commercially available kits. Individual susceptibility of peripheral blood lymphocytes (PBL) to glucocorticoids was also evaluated. Results Paradoxically, the patients with Bcc infection (n = 43) demonstrated better PFT outcomes and higher Body Mass Indexes (BMI) compared to those of uninfected children (n = 52). In the same time multiple antibiotic allergies as well as glucose tolerance abnormalities were more common among the patients of Bcc group. We did not find significant differences for in situ inflammatory markers between CF patients with and without Bcc infection. At once marked reduction of circulating IL-17 and TGF-β1 as well as increased sensitivity of PBL to glucocorticoids have been revealed in the infected group. Conclusion The paradoxical association of chronic Bcc colonization with better PFT outcomes, higher BMI and down-regulated systemic inflammatory markers in CF children may relate to more aggressive antibiotic therapy as well as Bcc-induced alterations of host inflammatory response. Further studies are needed to better understand the specific nature of the host–pathogen interactions during chronic Bcc colonization.