Cystic fibrosis: A system for assessing and predicting progression

Robert H. Cleveland, Ariane Staub Neish, David Zurakowski, David P. Nichols, Mary Ellen B. Wohl, Andrew A. Colin

Research output: Contribution to journalArticlepeer-review

30 Scopus citations


OBJECTIVE. This study presents a radiography-based database scoring changes 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 treatment. The data may also be used to compare individuals with their age-matched cohorts with cystic fibrosis. MATERIALS AND METHODS. From 230 patients, 3038 chest radiographs were scored using the Brasfield system. The scores from radiographs from all the patients were individually 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 population. RESULTS. We found high inter- and intraobserve reliability. The difference between the observers averaged 1.3 Brasfield points, the scale of which ranges up to 25 points. The age-based severity curve was presented as mean Brasfield scores versus age (birth > 30 years) plotted with 95% confidence limits; the curve was also plotted in percentiles. 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 pulmonary function studies, which are functional. Thus the age-based severity curve provides an additional, independent basis for comparison between groups and individuals. It may be used for the initial assessment of 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 treatments in groups of patients with cystic fibrosis.

Original languageEnglish (US)
Pages (from-to)1067-1072
Number of pages6
JournalAmerican Journal of Roentgenology
Issue number4
StatePublished - Apr 1998
Externally publishedYes

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging


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