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New study brings hope for preventing lung disease in children with cystic fibrosisResearchers at Perth's Telethon Institute are one step closer to preventing serious lung disease which is the main cause of suffering in cystic fibrosis.
Research
Using syringe infusion pumps Springfusors for the administration of intravenous antibiotics for children with CF at PCH (SIPS-CF)André Schultz MBChB, PhD, FRACP Program Head, Respiratory Health RFA Program Head, Respiratory Health RFA Prof André Schultz is the Program Head of

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Treat early to protect lungsEarly intervention is being touted as the key to preventing lung damage in children with cystic fibrosis.

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Cystic fibrosis research a Eureka Prize finalistA world-leading cystic fibrosis research program, based at The Kids Research Institute Australia, is a finalist in the 2015 Australian Museum Eureka Prizes.
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Healthy lungs, healthy lifeThe lungs are one of the last organs in the body to develop as a baby grows. They're also one of the most important.

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$3.4mill to improve treatment management of cystic fibrosisThe Kids researchers have been awarded over $3.4 million for a new trial to pioneer improved ways for managing cystic fibrosis (‘CF’).
Research
Airway macrophages display decreased expression of receptors mediating and regulating scavenging in early cystic fibrosis lung diseaseCystic fibrosis (CF) airway disease is characterized by chronic inflammation, featuring neutrophil influx to the lumen. Airway macrophages (AMs) can promote both inflammation and resolution, and are thus critical to maintaining and restoring homeostasis. CF AM functions, specifically scavenging activity and resolution of inflammation, have been shown to be impaired, yet underlying processes remain unknown.
Research
Prevalence of tracheobronchomalacia is higher than previously reported in children with cystic fibrosisTracheobronchomalacia (TBM) is estimated to be present in 1 in 2100 children. Previous reports suggest the prevalence is higher in children with cystic fibrosis (CF). This has clinical implications with potential to influence airway clearance and lung health.
Research
BEAT CF pulmonary exacerbations core protocol for evaluating the management of pulmonary exacerbations in people with cystic fibrosisCystic fibrosis (CF) is a rare, inherited, life-limiting condition predominantly affecting the lungs, for which there is no cure. The disease is characterized by recurrent pulmonary exacerbations (PEx), which are thought to drive progressive lung damage. Management of these episodes is complex and generally involves multiple interventions targeting different aspects of disease. The emergence of innovative trials and use of Bayesian statistical methods has created renewed opportunities for studying heterogeneous populations in rare diseases.
Research
The effect of azithromycin on structural lung disease in infants with cystic fibrosis (COMBAT CF): a phase 3, randomised, double-blind, placebo-controlled clinical trialStructural lung disease and neutrophil-dominated airway inflammation is present from 3 months of age in children diagnosed with cystic fibrosis after newborn screening. We hypothesised that azithromycin, given three times weekly to infants with cystic fibrosis from diagnosis until age 36 months, would reduce the extent of structural lung disease as captured on chest CT scans.