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Title: Magnetic resonance imaging and multiple breath washout to assess ventilation in cystic fibrosis
Author: Smith, Laurie
Awarding Body: University of Sheffield
Current Institution: University of Sheffield
Date of Award: 2019
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Patients with CF are now progressively living longer than previously possible. This is largely down to significant improvements in treatments as well as factors such as the introduction of newborn screening allowing treatments to be started earlier. CFTR modulators potentially will progress the life expectancy even further, especially with recent encouraging data on triple therapy. Despite this, lung disease remains the primary cause of morbidity and mortality in patients with CF. The annual deterioration of FEV1 is now typically small, which makes it insensitive to detect small changes in lung function. More so than ever, sensitive methods of assessment of lung disease are required to effectively intervene before disease progression becomes advanced. Clinical trials also require sensitive assessment methods in order to assess the efficacy of the intervention. FEV1 is no longer necessarily suitable because more and more patients have normal FEV1 for significantly longer periods of time, FEV1 currently therefore requires large sample sizes to demonstrate the true effect of the intervention. MBW and MRI are two such promising methods of sensitively assessing lung function. Both methods are more sensitive than FEV1 at detecting early lung disease, however MBW does not always have a predictable outcome in patients with more severe disease, and is a simple summary measure of overall ventilation efficiency. Ventilation and 1H MRI are promising radiation free imaging methods of both the function and structure of the lungs. Compared to lung function tests, MRI has the advantage of being able to visualise and quantify regional lung disease, which may allow for the most sensitive detection of early lung disease and potentially for targeted treatments. The utility of MBW in mild disease is well understood, but this is less so in patients with more severe disease. By performing MBW alongside ventilation MRI the impact of lung disease on MBW may be better understood. Ventilation MRI in CF lacks longitudinal data to understand how it may track CF lung disease and whether it is more sensitive than current methods for detecting changes in lung function.
Supervisor: Wild, Jim ; Horsley, Alex Sponsor: Not available
Qualification Name: Thesis (Ph.D.) Qualification Level: Doctoral
EThOS ID:  DOI: Not available