Pim A. de Jong, MSc Mark D. Ottink, MD Simon G. F. Robben, MD, PhD Maarten H. Lequin, MD, PhD Wim C. J. Hop, PhD Johan J. E. Hendriks, MD, PhD Peter D. Pare ´, MD Harm A. W. M. Tiddens, MD, PhD Index terms: Computed tomography (CT), in infants and children, 60.1211, 60.12118 Computed tomography (CT), thin- section, 60.12118 Fibrosis, cystic, 60.252 Lung, CT, 60.1211, 60.12118 Lung, function Published online before print 10.1148/radiol.2312021393 Radiology 2004; 231:434 – 439 Abbreviations: FEF 25%–75% = forced expiratory flow between 25% and 75% of expiratory vital capacity FEV 1 = forced expiratory volume in 1 second FVC = forced vital capacity PFT = pulmonary function test Raw = airway resistance RV = residual volume TLC = total lung capacity 1 From the Depts of Paediatric Pulmonology (P.A.d.J., H.A.W.M.T.) and Paediatric Radiol- ogy (M.H.L.), Erasmus Med Ctr Rotterdam, Sophia Children’s Hosp, Dr Molewaterplein 60, 3015 GJ Rotterdam, the Netherlands; Dept of Epidemiology and Biostatistics (W.C.J.H.), Erasmus Med Ctr Rotterdam, the Netherlands; Depts of Paediatric Pulmonology (M.D.O., J.J.E.H.) and Radiology (S.G.F.R.), Univ Hosp Maastricht, the Netherlands; and Univ of British Columbia, McDonald Research Lab and iCAPTURE Ctr, St Paul’s Hosp, Van- couver, Canada (P.D.P.). Received Oct 29, 2002; revision requested Jan 9, 2003; final revision received Sep 10; accepted Sep 29. Address correspondence to H.A.W.M.T. (e-mail: h.tiddens@erasmusmc.nl ). See also the editorial by Brody in this issue. Author contributions: Guarantors of integrity of entire study, P.A.d.J., H.A.W.M.T.; study concepts and de- sign, P.A.d.J., M.H.L., S.G.F.R., W.C.J.H., H.A.W.M.T.; literature research, P.A.d.J., H.A.W.M.T.; clinical studies, H.A.W.M.T., S.G.F.R., M.H.L.; data acquisition, P.A.d.J., M.D.O., S.G.F.R.; data analysis/interpreta- tion, P.A.d.J., M.D.O., J.J.E.H., S.G.F.R., H.A.W.M.T., W.C.J.H., P.D.P.; statistical anal- ysis, P.A.d.J., M.D.O., H.A.W.M.T., W.C.J.H.; manuscript preparation, P.A.d.J., M.D.O., J.J.E.H., H.A.W.M.T.; manuscript definition of intellectual content, P.D.P., H.A.W.M.T., P.A.d.J.; manuscript editing, H.A.W.M.T., J.J.E.H., P.A.d.J., P.D.P., M.H.L., S.G.F.R., M.D.O.; manuscript revision/review and final version approval, all authors © RSNA, 2004 Pulmonary Disease Assessment in Cystic Fibrosis: Comparison of CT Scoring Systems and Value of Bronchial and Arterial Dimension Measurements 1 PURPOSE: To retrospectively compare thin-section computed tomographic (CT) scores obtained with five scoring systems for assessment of pulmonary disease in children with cystic fibrosis and to determine additional value of bronchial and arterial dimension measurements. MATERIALS AND METHODS: Scores obtained with five thin-section CT scoring systems were compared. A score of 0 indicated the absence of abnormalities; a higher score meant that more structural abnormalities were seen. Three observers assigned scores and then reassigned scores after intervals varying from 1–2 weeks to 1–2 months at review of thin-section CT scans obtained in 25 children with cystic fibrosis. Interobserver and intraobserver reliability was calculated with intraclass correlation coefficients. Quantitative measurements of bronchial and arterial dimen- sions were obtained. Thin-section CT scores were correlated (Spearman correlation) with bronchial and arterial dimensions and with results of pulmonary function tests (PFTs), such as forced expiratory volume in 1 second (FEV 1 ). RESULTS: Scores with all five scoring systems were reproducible, with intraclass correlation coefficients of 0.74 and higher (P  .05), and showed significant correlations with FEV 1 (R =-0.73 to -0.69, P  .01). Ratio of bronchial diameter to accompanying pulmonary arterial diameter was correlated with thin-section CT scores but not with FEV 1 . Ratio of bronchial wall thickness to accompanying pul- monary arterial diameter was not correlated with thin-section CT scores or PFT results. CONCLUSION: Thin-section CT scores were reproducible and were correlated with PFT results. Measurements of bronchial dimensions were not significantly related to scores or PFT results. © RSNA, 2004 Cystic fibrosis is the most frequently inherited autosomal recessive disease in whites, with an incidence of one in 3,600 in the Dutch population. Cystic fibrosis is a lethal disease; when it was described by Fanconi (1) and Andersen (2) more than 60 years ago, the median survival was less than 1 year. Presently, median survival is 32.3 years and is increasing (3). Despite increased longevity, pulmonary dysfunction causes major morbidity in cystic fibrosis, and more than 90% of the mortality is caused by pulmonary complications (4). It is therefore critically important to monitor progression of lung disease for clinical treat- ment and to evaluate new treatments. The standard for assessment of lung disease in cystic fibrosis is pulmonary function tests (PFTs); however, conventional PFTs are not very sensitive in the detection of early lung damage (5). In addition, the conventional PFTs are reliable only in children older than 5 years. There is evidence that pulmonary disease starts 434 R adiology