Authors:
Rosenow T, Oudraad MC, Murray CP, Turkovic L, Kuo W, de Bruijne M, ... Stick SM.
Authors notes:
American journal of respiratory and critical care medicine. 2015;191(10):1158-65.
Keywords:
clinical trials as topic, medical imaging, pediatrics, Chest computed tomography, cystic fibrosis
Abstract:
RATIONALE: Chest computed tomography (CT) is the gold standard for demonstrating cystic fibrosis (CF) airway disease.
However, there are no standardized outcome measures appropriate for children younger than 6 years.
OBJECTIVES: We developed the Perth-Rotterdam Annotated Grid Morphometric Analysis for CF (PRAGMA-CF), a quantitative measure of airway disease, and compared it with the commonly used CF-CT scoring method.
METHODS: CT scans from the Australian Respiratory Early Surveillance Team for CF (AREST CF) cohort in Western Australia were included.
PRAGMA-CF was performed by annotating a grid overlaid on 10 axial slices for the presence of bronchiectasis, mucous plugging, or other airway abnormalities (inspiratory scans) and trapped air (expiratory scans).
The separate proportions of total disease (%Dis), bronchiectasis (%Bx), and trapped air (%TA) were determined.
Thirty scans were used for observer reliability, and 30 paired scans obtained at 1 and 3 years old were used for comparison with a validated standard and biologic plausibility.
MEASUREMENTS AND MAIN RESULTS: Intraobserver, intraclass correlation coefficients for %Dis, %Bx, and %TA were 0.93, 0.93, and 0.96, respectively.
The change in %Dis and %Bx with PRAGMA-CF was related to neutrophil elastase presence at age 3, whereas only the change in bronchiectasis score was related to neutrophil elastase with CF-CT.
Sample-size calculations for various effect sizes are presented.
CONCLUSIONS: PRAGMA-CF is a sensitive and reproducible outcome measure for assessing the extent of lung disease in very young children with CF.