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Early diagnosis and treatment in Cystic Fibrosis (CF) has improved health outcomes for patients, but lung disease driven by inflammation still causes the majority of CF-related complications, with inflammation often present even without bacterial infections. Recent research has suggested thick mucus present in CF airways reduces oxygen in airway cells and results in inflammation. This study asks two important questions: Does lung inflammation caused by mucus blockages in the airway occur early in life in young children with CF? And is it related to known CF inflammatory markers and lung damage seen in the CF lung? We obtained samples from young children with and without lung infections and measured specific inflammatory markers such as interleukin-1alpha and investigated relationships between these markers and lung damage measured using lung imaging. We found relationships between lung damage in children without infection and interleukin-1alpha, suggesting interleukin-1alpha has a role in lung damage in CF when there is no bacterial infection, and supports earlier research performed in animal models. However the relationships between inflammatory markers and lung damage doesn’t mean it is directly driven by interleukin-1alpha, but does provide good evidence that this is an area that we should investigate further using human airway cells to try and figure out what drives this specific early inflammation in the airways of young children with CF.

Authors: Samuel T. Montgomery, A. Susanne Dittrich, Luke W. Garratt, Lidija Turkovic, Dario L. Frey, Stephen M. Stick, Marcus A. Mall, Anthony Kicic, AREST CF

Published in the Journal of Cystic Fibrosis in May 2018.