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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 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.
Research
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.
Research
Improving screening in a paediatric cohort for cystic fibrosis-related diabetes: A quality improvement projectAndré Schultz MBChB, PhD, FRACP Head, BREATH Team Head, BREATH Team Prof André Schultz is the Head, BREATH Team at The Kids Research Institute
Research
Progress in Model Systems of Cystic Fibrosis Mucosal Inflammation to Understand Aberrant Neutrophil ActivityHere we examine the latest findings of neutrophils in pediatric CF lung disease and proposed mechanisms of their pathogenicity

News & Events
New drug offers hope for people living with cystic fibrosisA promising new treatment pioneered in Western Australia for people with cystic fibrosis has commenced testing in a clinical trial in the United States and Australia.

News & Events
MRCF launches Perth-based biotech developing new treatment for Cystic FibrosisA The Kids Research Institute Australia spin-off company has received $20 million from the Medical Research Commercialisation Fund to develop a promising new therapy for the treatment of Cystic Fibrosis.

News & Events
Child health a focus in national research grantsThe Kids Research Institute Australia researchers have been awarded more than $8 million in prestigious project grants from the NHMRC.

News & Events
International clinical trial reduced lung inflammation in young kids with cystic fibrosisPromising results from an Australian-led clinical trial could drastically change the way we care for young children with cystic fibrosis (CF).
Research
Single-breath washout and association with structural lung disease in children with cystic fibrosisAcinar ventilation inhomogeneity measured by single-breath washout was not associated with structural lung disease on CT