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PREDICT CF: Assessing the role of lung clearance index in predicting disease progression in children with cystic fibrosis

Investigators: Alana Harper, Graham Hall, Joseph Bartlett, Luke Berry, Nat Eiffler, Rebecca Langford, Yuliya Karpievitch, Stephen Stick, Tim Rosenow, Anoop Ramgolam, Elizabeth Smith, Jasmine Grdosic

External collaborators: Sarath Ranganathan (Murdoch Children's Research Institute)

There is increasing evidence that the assessment of ventilation distribution using the multiple breath washout (MBW) technique is sensitive to changes in disease status of children with cystic fibrosis. The most common reported measure from MBW is the Lung Clearance Index (LCI). The LCI provides a quantitative assessment of the missing of gases within the lung. MBW can be carried out in children as young at 3 years old and has been shown to be a may be a more sensitive measure of disease progression in young children. This study aims to conduct a full audit of all research and clinical data for all children with repeated multiple measures of LCI and clinical outcomes. By doing this the primary objective will be to relate longitudinal changes in LCI to disease severity and progression in children with CF.

This research is funded by Vertex Pharmaceuticals.