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Signal-correction errors in the EasyOne Pro LAB multiple-breath washout device significantly impact outcomes in children and adultsMultiple-breath washout (MBW) is an established technique to assess functional residual capacity (FRC) and ventilation inhomogeneity in the lung. Indirect calculation of nitrogen concentration requires accurate measurement of gas concentrations.
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Pulmonary bacteriophage and cystic fibrosis airway mucus: friends or foes?For those born with cystic fibrosis (CF), hyper-concentrated mucus with a dysfunctional structure significantly impacts CF airways, providing a perfect environment for bacterial colonization and subsequent chronic infection. Early treatment with antibiotics limits the prevalence of bacterial pathogens but permanently alters the CF airway microenvironment, resulting in antibiotic resistance and other long-term consequences.
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Detection of bile acids in bronchoalveolar lavage fluid defines the inflammatory and microbial landscape of the lower airways in infants with cystic fibrosisCystic Fibrosis (CF) is a genetic condition characterized by neutrophilic inflammation and recurrent infection of the airways. How these processes are initiated and perpetuated in CF remains largely unknown. We have demonstrated a link between the intestinal microbiota-related metabolites bile acids and inflammation in the bronchoalveolar lavage fluid from children with stable CF lung disease.
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A near-complete genome of the uncultured Staphylococcus aureus phage COMBAT-CF_PAR1 isolated from the lungs of an infant with cystic fibrosisIn cystic fibrosis, bacteria–bacteriophage interaction in the lower airways is poorly understood. We present the near-complete genome of the uncultured Siphovirus-like bacteriophage, Staphylococcus aureus phage COMBAT-CF_PAR1, isolated from the lower airways. The genome spans 41,510 bp with 33.45% guanine–cytosine content and contains 65 open reading frames.
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Respiratory tract exacerbations revisited: Ventilation, inflammation, perfusion, and structure (VIPS) monitoring to redefine treatmentFor cystic fibrosis (CF) patients older than 6 years there are convincing data that suggest respiratory tract exacerbations (RTE) play an important role in...
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Reversibility of trapped air on chest computed tomography in cystic fibrosis patientsTo investigate changes in trapped air volume and distribution over time and compare computed tomography (CT) with pulmonary function tests for determining...
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Lung Clearance Index and Structural Lung Disease on Computed Tomography in Early Cystic FibrosisLung clearance index may be a useful surveillance tool to monitor structural lung disease in preschool and school-age children with cystic fibrosis
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Impact of lung disease on respiratory impedance in young children with cystic fibrosisThe present study investigated whether lung function measured by forced oscillation technique would be impaired in the presence of infection,...
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Changes in airway inflammation with pseudomonas eradication in early cystic fibrosisNeutrophil elastase is a significant risk factor for structural lung disease in cystic fibrosis, and Pseudomonas aeruginosa airway infection is linked with neutrophilic inflammation and substantial respiratory morbidity. We aimed to evaluate how neutrophil elastase (NE) activity changes after P. aeruginosa eradication and influences early disease outcomes. We assessed participants in the AREST CF cohort between 2000 and 2018 who had P. aeruginosa cultured from their routine annual bronchoalveolar lavage (BAL) fluid and who underwent eradication treatment and a post eradication BAL. Factors associated with persistent P. aeruginosa infection, persistent neutrophilic inflammation following eradication and worse structural lung disease one year post-eradication were evaluated.
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A Small Device May Deliver King-Sized Solutions for Patients With an Exacerbation of Cystic FibrosisThe aim is to examine whether using a portable spring-infusor device to deliver antibiotics compared with a standard infusion pump (SIP) translated to (i) improve health outcomes, (ii) reduce the length of stay (LoS), and (iii) reduce cost for treatment of exacerbations of cystic fibrosis.