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New study recommends changes to cystic fibrosis monitoring in young children

A new Australian study that looked at the long term impacts of early lung infections in young kids with cystic fibrosis has recommended changes to monitoring

Research

Survival of people with cystic fibrosis in Australia

Survival statistics, estimated using data from national cystic fibrosis (CF) registries, inform the CF community and monitor disease progression. This study aimed to estimate survival among people with CF in Australia and to identify factors associated with survival.

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Detection of biofilm in bronchoalveolar lavage from children with non-cystic fibrosis bronchiectasis

The presence of Pseudomonas aeruginosa biofilms in lower airway specimens from cystic fibrosis (CF) patients is well established.

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Determinants of culture success in an airway epithelium sampling program of young children with cystic fibrosis

Determinants of culture success through retrospective analysis of a program of routinely brushing children with Cystic Fibrosis airway disease

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Assessment of early bronchiectasis in young children with cystic fibrosis is dependent on lung volume

The aim of this study was to determine whether assessment of early CT scan-detected bronchiectasis in young children with cystic fibrosis (CF) depends on...

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Novel end points for clinical trials in young children with cystic fibrosis

Cystic fibrosis (CF) lung disease commences early in the disease progression and is the most common cause of mortality.

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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.

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Stability of interleukin 8 and neutrophil elastase in bronchoalveolar lavage fluid following long-term storage

Interleukin-8 (IL-8) and neutrophil elastase (NE) are commonly measured markers of inflammation in bronchoalveolar lavage (BAL) fluid from patients with cystic

Research

Cystic Fibrosis Early Surveillance Program

Researchers are able to track the progress of lung disease through a comprehensive longitudinal set of biological samples, images and data archives.

Research

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.