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Early detection of lung function abnormalities in young children with cystic fibrosis

In this review, we have examined the role of lung function testing in infants and preschool children with CF.

Evolution of pulmonary inflammation and nutritional status in infants and young children with cystic fibrosis

Improved nutrition is the major proven benefit of newborn screening programmes for cystic fibrosis (CF) and is associated with better clinical outcomes.

Inflammatory responses to individual microorganisms in the lungs of children with cystic fibrosis

We hypothesized that the inflammatory response in the lungs of children with cystic fibrosis (CF) would vary with the type of infecting organism, being...

Cyanide in bronchoalveolar lavage is not diagnostic for Pseudomonas aeruginosa in children with cystic fibrosis

Early detection of the cyanobacterium Pseudomonas aeruginosa in the lungs of young children with cystic fibrosis (CF) is considered the key to delaying...

Promising results for new Western Australian-pioneered cystic fibrosis drug

Children with cystic fibrosis will be included in the next phase of a clinical trial of a promising new treatment pioneered in Western Australia aimed at boosting their immune responses to infections. Originally developed by researchers at The Kids Research Institute Australia and Perth Children’s Hospital.

Foundations of Lung Disease

The Foundations of Lung Disease Team is focused on improving the diagnosis, treatment, and lifelong care of childhood lung disease.

Helpful resources for individuals born preterm

We’ve heard from families that trustworthy information about preterm-associated lung disease is difficult to find. In response, we’ve created resources to empower families with the knowledge they need to manage these challenges.

A pilot study of disease related education and psychotherapeutic support for unresolved grief in parents of children with CF

Diagnosis of chronic disease in a child can result in unresolved grief (UG) in parents. This study aimed to evaluate the efficacy of psychological insight-oriented therapy (IOT) as a treatment for UG compared to disease related education in parents of children with cystic fibrosis. Sequence of delivery, first IOT then disease related education (or vice versa) was also examined, to let all participants experience both interventions.

Mucus and mucus flake composition and abundance reflect inflammatory and infection status in cystic fibrosis

Mucus hyperconcentration in cystic fibrosis (CF) lung disease is marked by increases in both mucin and DNA concentration. Additionally, it has been shown that half of the mucins present in bronchial alveolar lavage fluid from preschool-aged CF patients are present in as non-swellable mucus flakes.

SLC6A14 Impacts Cystic Fibrosis Lung Disease Severity via mTOR and Epithelial Repair Modulation

Cystic fibrosis (CF), due to pathogenic variants in CFTR gene, is associated with chronic infection/inflammation responsible for airway epithelium alteration and lung function decline. Modifier genes induce phenotype variability between people with CF (pwCF) carrying the same CFTR variants. Among these, the gene encoding for the amino acid transporter SLC6A14 has been associated with lung disease severity and age of primary airway infection by the bacteria Pseudomonas aeruginosa.