thesis
Monitoring of Cystic Fibrosis Lung Disease Using Computed Tomography
- Publication date
- 14 December 2005
- Publisher
- For clinical management of cystic fibrosis (CF) lung disease
to be effective, onset and worsening of lung abnormalities should be closely
monitored. Pulmonary function tests (PFTs) are currently the gold standard to
monitor CF lung disease. Lung structure can be more sensitively monitored using
computed tomography (CT) rather than chest radiography. Firstly, we compared in
two pediatric cohorts the sensitivities of CT and PFTs to detect onset and
worsening of CF lung disease. We showed that five published CT scoring systems
are comparable and have good intra- and interobserver agreement. Secondly, we
showed that CT scoring systems and quantitative CT-measurements of airway wall
thickening and bronchiectasis are more sensitive to detect the start and
worsening of CF lung disease than are PFTs. Bronchiectasis-score worsened most
in children and the worsening remained undetected by the PFTs and the
quantitative measurements. Quantitative CT-measurements of air!
way wall thickening worsened significantly, whereas PFTs and airway wall
thickness measured by scoring remained unchanged. Thirdly, we developed a
computational model to study radiation risks associated with CT scanning in CF.
Risks from lifelong biennial CT scanning in CF were found to be acceptably low
given the currently reduced life expectancy. Finally we provided normal
CT-values of lung parenchyma and airway wall and lumen that can be used to study
lung growth aberrations due to CF. Our data support routine CT scanning to
monitor CF lung disease. In addition, bronchiectasis-score and quantitatively
measured airway wall thickening may be useful surrogate endpoints for clinical
trials in CF.