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Research

Chest imaging in cystic fibrosis studies: What counts, and can be counted?

The aim of this study is to characterize the role of currently available CT and MRI markers in clinical studies, and to discuss challenges with CF studies.

Research

Air pollution during pregnancy and lung development in the child

Air pollution exposure has increased in recent years and there is evidence that exposure to particulate matter can lead to adverse respiratory outcomes.

Research

Clinical investigation of respiratory system admittance in preschool children

We compared the ability of Ars, to standard oscillatory outcomes, to determine respiratory disease and differentiate responses to inhaled bronchial challenges.

Research

Air Trapping on Chest CT Is Associated with Worse Ventilation Distribution in Infants with Cystic Fibrosis

In school-aged children with cystic fibrosis (CF) structural lung damage assessed using chest CT is associated with abnormal ventilation distribution.

Research

Exciting new clinical trials in cystic fibrosis: Infants need not apply

The recent announcement of the negative results of the TIGER- 2 phase 3 study of denufosol tetrasodium

News & Events

Very preterm babies at risk of declining lung function throughout childhood

A The Kids Research Institute Australia study published in The Lancet Child & Adolescent Health has found that survivors of very preterm birth face declining lung function

Research

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.

Research

Genome analysis and phenotypic characterization of Halomonas hibernica isolated from a traditional food process with novel quorum quenching and catalase activities

Traditional food processes can utilize bacteria to promote positive organoleptic qualities and increase shelf life. Wiltshire curing has a vital bacterial component that has not been fully investigated from a microbial perspective.

Research

Macrophage PD-1 associates with neutrophilia and reduced bacterial killing in early cystic fibrosis airway disease

Macrophages are the major resident immune cells in human airways coordinating responses to infection and injury. In cystic fibrosis, neutrophils are recruited to the airways shortly after birth, and actively exocytose damaging enzymes prior to chronic infection, suggesting a potential defect in macrophage immunomodulatory function.

Research

EVC-EVC2 complex stability and ciliary targeting are regulated by modification with ubiquitin and SUMO

Ellis van Creveld syndrome and Weyers acrofacial dysostosis are two rare genetic diseases affecting skeletal development. They are both ciliopathies, as they are due to malfunction of primary cilia, microtubule-based plasma membrane protrusions that function as cellular antennae and are required for Hedgehog signaling, a key pathway during skeletal morphogenesis.