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Research

Single-breath washout and association with structural lung disease in children with cystic fibrosis

Acinar ventilation inhomogeneity measured by single-breath washout was not associated with structural lung disease on CT

Research

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.

Research

Detection of bile acids in bronchoalveolar lavage fluid defines the inflammatory and microbial landscape of the lower airways in infants with cystic fibrosis

Cystic 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.

Research

Lung Clearance Index and Structural Lung Disease on Computed Tomography in Early Cystic Fibrosis

Lung clearance index may be a useful surveillance tool to monitor structural lung disease in preschool and school-age children with cystic fibrosis

Research

Impact of lung disease on respiratory impedance in young children with cystic fibrosis

The present study investigated whether lung function measured by forced oscillation technique would be impaired in the presence of infection,...

Research

Respiratory tract exacerbations revisited: Ventilation, inflammation, perfusion, and structure (VIPS) monitoring to redefine treatment

For cystic fibrosis (CF) patients older than 6 years there are convincing data that suggest respiratory tract exacerbations (RTE) play an important role in...

Research

Reversibility of trapped air on chest computed tomography in cystic fibrosis patients

To investigate changes in trapped air volume and distribution over time and compare computed tomography (CT) with pulmonary function tests for determining...

Research

Early cystic fibrosis lung disease

This paper is about lung disease in patients with cyctic Fibrosis and prevention strategies to slow the onset of lung disease.

Research

Parental experiences of early pulmonary surveillance for children with cystic fibrosis: A research proposal for improved family psychosocial outcomes

The proposed study intends to explore parental experiences, including coping, related to their child's involvement in the early surveillance program.

Research

Innate inflammatory responses of pediatric cystic fibrosis airway epithelial cells: Effects of nonviral and viral stimulation

There is controversy regarding whether cystic fibrosis (CF) airway epithelial cells (AECs) are intrinsically proinflammatory.