CYSTIC-FIBROSIS - A SYSTEM FOR ASSESSING AND PREDICTING PROGRESSION

OBJECTIVE. This study presents a radiography-based database scoring ch anges over time in a large population of patients with cystic fibrosis . The purpose of this database is to provide comparison for groups of patients undergoing experimental treatment to assess effect of the tre atment. The data may also be used to compare individuals with their ag e-matched cohorts with cystic fibrosis. MATERIALS AND METHODS. From 23 0 patients, 3038 chest radiographs were scored using the Brasfield sys tem. The scores from radiographs from all the patients were individual ly plotted for age, and a single age-based severity curve was created. The age-based severity curve was compared with similar curves derived from pulmonary function studies of a subset of the same patient popul ation. RESULTS. We found high inter-and intraobserver reliability. The difference between the observers averaged 1.3 Brasfield points, the s cale of which ranges up to 25 points. The age-based severity curve was presented as mean Brasfield scores versus age (birth to >30 years) pl otted with 95% confidence limits; the curve was also plotted in percen tiles. The rate of decline of this curve was similar to the decline of pulmonary function studies in this patient population.CONCLUSION. The age-based curve, a structural anatomic parameter, differs from pulmon ary function studies, which are functional. Thus the age-based severit y curve provides an additional, independent basis for comparison betwe en groups and individuals. It may be used for the initial assessment o f lung disease and for gauging and predicting the rate of decline. The curve may be used as a long-range outcome criterion to evaluate new t reatments in groups of patients with cystic fibrosis.