NTM infection is of increasing prevalence in the UK paediatric CF population. This study highlights the urgent need for work to establish effective treatment and prevention strategies for NTM infection in young people with CF.

BackgroundChronic lung infection with Pseudomonas aeruginosa remains a major cause of mortality and morbidity among individuals with CF. Expression of mediators promoting recruitment and differentiation of B cells, or supporting antibody production is poorly understood yet could be key to controlling infection.MethodsBAFF was measured in BAL from children with CF, both with and without P. aeruginosa, and controls. Mice were intra-nasally infected with P. aeruginosa strain LESB65 for up to 7 days. Cellular infiltration and expression of B cell chemoattractants and B cell differentiation factor, BAFF were measured in lung tissue.ResultsBAFF expression was elevated in both P. aeruginosa negative and positive CF patients and in P. aeruginosa infected mice post infection. Expression of the B cell chemoattractants CXCL13, CCL19 and CCL21 increased progressively post infection.ConclusionsIn a mouse model, infection with P. aeruginosa was associated with elevated expression of BAFF and other B cell chemoattractants suggesting a role for airway B cell recruitment and differentiation in the local adaptive immune response to P. aeruginosa. The paediatric CF airway, irrespective of pseudomonal infection, was found to be associated with an elevated level of BAFF implying that BAFF expression is not specific to pseudomonas infection and may be a feature of the CF airway. Despite the observed presence of a potent B cell activator, chronic colonisation is common suggesting that this response is ineffective.

Objective To assess the impact of breathing retraining on asthma symptoms and dysfunctional breathing (DB) in children. Breathing retraining can improve DB but there is a lack of evidence in pediatrics. Methods Participants attended outpatient physiotherapy appointments and received individually tailored interventions, particularly Buteyko breathing techniques. The primary outcome was the change in the Asthma Control Test (ACT) score or change in childhood ACT (CACT) score from first to final appointment. The ACT and CACT are validated in children more than or equal to 12 years and children aged 4 to 11, respectively. The secondary outcome measure was the change in Nijmegen Questionnaire (NQ) score from first to the final appointment (score range, 0‐64) with a score of more than or equal to 23 indicating DB symptoms. Results One hundred and sixty‐nine children with asthma attended and completed a mean of six physiotherapy sessions, over a mean of 15 weeks. Patients were aged 2 to 18, mean 10 years. Fifty‐five patients were more than or equal to 12 years old and 114 were less than or equal to 11 years. One hundred and seven patients were receiving BTS/SIGN asthma guideline step 1 to 3 therapy and 62 were on step 4 to 5 therapy. The mean ACT score improved by 4.4 (P < 0.0001), the mean CACT score improved by 4.9 (P < 0.0001), and the mean NQ score change improved by −9.3 points (P < 0.0001). Conclusion In addition to standard medical therapy, individually tailored physiotherapy interventions improved asthma control and DB in children on all levels of asthma treatment. A randomized controlled study is required to determine whether these improvements are due to the intervention.