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Background: Chest computerized tomography (CT) scores are associated with the frequency of future pulmonary exacerbations in people with cystic fibrosis (CF). However, cut-off values to identify children with mild lung disease with different risks for frequent future pulmonary exacerbations have not been identified., Methods: Chest CT scans were assessed using the Brody score for participants of the Pulmozyme Early Intervention Trial (PEIT) and Wisconsin Randomized Clinical Trial of CF Newborn Screening (WI RCT). We determined the area under the receiver operating characteristic (ROC) curve for Brody scores and forced expiratory volume in 1 s (FEV 1 ) to compare with the frequency of pulmonary exacerbations up to 10 years later., Results: There were 60 participants in the PEIT with mean (SD) age 10.6 (1.7) years at the time of the CT and 81 participants in the WI RCT with mean age 11.5 (3.0) years. The Brody score cut-off that best identified children at-risk for ≥0.3 annual pulmonary exacerbations was 3.6 in the PEIT and 2.1 in the WI RCT. There were no statistical differences between ROC curves for the Brody CT score and FEV 1 % predicted in either study (P ≥ 0.4)., Conclusions: CT score cut-off values that identify children with CF with mild lung disease at different risks for frequent pulmonary exacerbations over an extended follow up period are similar in separate cohorts. Brody scores and FEV 1 % predicted have similar abilities to identify these children, suggesting that FEV 1 % predicted alone may be adequate for predicting future frequency of pulmonary exacerbations., (© 2018 Wiley Periodicals, Inc.)

Objective: To determine whether clinical scoring systems or physician gestalt can obviate the need for computed tomography (CT) in patients with possible appendicitis., Methods: Prospective, observational study of patients with abdominal pain at an academic emergency department (ED) from February 2012 to February 2014. Patients over 11 years old who had a CT ordered for possible appendicitis were eligible. All parameters needed to calculate the scores were recorded on standardised forms prior to CT. Physicians also estimated the likelihood of appendicitis. Test characteristics were calculated using clinical follow-up as the reference standard. Receiver operating characteristic curves were drawn., Results: Of the 287 patients (mean age (range), 31 (12-88) years; 60% women), the prevalence of appendicitis was 33%. The Alvarado score had a positive likelihood ratio (LR(+)) (95% CI) of 2.2 (1.7 to 3) and a negative likelihood ratio (LR(-)) of 0.6 (0.4 to 0.7). The modified Alvarado score (MAS) had LR(+) 2.4 (1.6 to 3.4) and LR(-) 0.7 (0.6 to 0.8). The Raja Isteri Pengiran Anak Saleha Appendicitis (RIPASA) score had LR(+) 1.3 (1.1 to 1.5) and LR(-) 0.5 (0.4 to 0.8). Physician-determined likelihood of appendicitis had LR(+) 1.3 (1.2 to 1.5) and LR(-) 0.3 (0.2 to 0.6). When combined with physician likelihoods, LR(+) and LR(-) was 3.67 and 0.48 (Alvarado), 2.33 and 0.45 (RIPASA), and 3.87 and 0.47 (MAS). The area under the curve was highest for physician-determined likelihood (0.72), but was not statistically significantly different from the clinical scores (RIPASA 0.67, Alvarado 0.72, MAS 0.7)., Conclusions: Clinical scoring systems performed equally well as physician gestalt in predicting appendicitis. These scores do not obviate the need for imaging for possible appendicitis when a physician deems it necessary., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/)

Rationale: Abnormalities on chest computed tomography (CT) in children with cystic fibrosis (CF) have been shown to correlate with short-term measures of lung disease. Chest CT scores offer promise as a potential surrogate end point in CF; however, there is limited information available on the ability of chest CT scores to predict future morbidity., Objectives: Determine whether chest CT scores are associated with the rate of pulmonary exacerbations over the next 10 years., Methods: Ten years of follow-up data were obtained from the CF Foundation Patient Registry for 60 children enrolled in the Pulmozyme Early Intervention Trial and who had chest CT scans at baseline., Measurements and Main Results: Multivariable Poisson regression was used to compare Brody CT scores and the number of pulmonary exacerbations in the following 10 years. At the time of the chest CT, the mean (SD) age was 10.6 (1.7) years. A 1-point increase in the Brody CT score was associated with an increase in the mean (95% confidence interval) rate of pulmonary exacerbations of 1.39 (1.15, 1.67) (P < 0.001). Brody CT scores were more strongly associated with the number of pulmonary exacerbations than FEV1 % predicted at the time of the chest CT (P = 0.037 by chi-square test)., Conclusions: There is a significant association between Brody CT scores and the rate of pulmonary exacerbations up to 10 years later. This association is stronger than for FEV1 obtained at the time of the CT, suggesting that chest CT scores offer improved ability to predict future outcomes.

Rationale: Most children with cystic fibrosis (CF) experience a slow decline in spirometry, although some children continue to be at risk for more significant lung disease progression. Chest computed tomography (CT) scans have been shown to be more sensitive to changes in lung disease than spirometry and may provide a means for predicting future lung disease progression., Objectives: We hypothesized that Brody chest CT scan scores obtained in 2000 in a prospectively monitored cohort of children with CF would be associated with the most recent measures of lung disease severity., Methods: Brody chest CT scan scores were calculated for 81 children enrolled in the Wisconsin CF Neonatal Screening Project. Multivariable linear regression was used to determine associations between Brody scores and the most recent (age 21 yr or June 30, 2010, whichever was later) measures of CF lung disease., Measurements and Main Results: The mean observation time after the chest CT scan was 7.5 years. Brody chest CT scan scores were significantly associated with the most recent measures of spirometry (P < 0.001) and Wisconsin and Brasfield chest radiograph scores (P < 0.001). The strength of this association was much stronger than spirometry obtained near the time of the chest CT scan (P < 0.01) but not chest radiograph scores., Conclusions: Chest CT scan scores are associated with future lung disease severity, and quantitative chest imaging(chest CT scan and chest radiograph scores) is more strongly associated with future lung disease severity than measures of spirometry. These findings may help clinicians identify patients at risk of future lung disease progression.

Purpose: To correlate the severity of bronchiectasis in children with cystic fibrosis with clinical and microbiologic variables in order to clarify risk factors for the development of irreversible lung disease., Materials and Methods: After institutional review board approval and parental informed consents were obtained, a HIPAA-compliant longitudinal epidemiologic evaluation was performed in patients with cystic fibrosis who were enrolled in the Wisconsin trial of newborn screening from 1985 to 2009. Thin-section chest computed tomography (CT) was used in a prospective cross-sectional design to study patients ranging in age from 6.6 to 17.6 years (mean, 11.5 years). Thin-section CT scores were determined objectively on coded images by multiple raters in a standardized fashion. Microbiologic data were obtained by means of culture of respiratory secretions by using methods for differentiation of Pseudomonas aeruginosa (PA) as either nonmucoid or mucoid., Results: Eighty-three percent of patients (68 of 82) showed bronchiectasis of varying severity. Of 12 potential risk factors, only respiratory infection with mucoid PA correlated significantly with bronchiectasis (P = .041)., Conclusion: The severity of bronchiectasis in children with cystic fibrosis is significantly related to respiratory infection with mucoid PA; attempts to prevent bronchiectasis should include reducing exposure to and early eradication of PA.

Introduction: Computerized tomography (CT) scanning shows promise as an outcome surrogate for cystic fibrosis (CF) lung disease progression. The scoring system used to convert the CT image to numeric data is an essential determinant of the performance of CT scanning., Methods: Three radiologists independently scored 16 high-resolution CT scans performed on children in the Wisconsin CF Neonatal Screening Project. The test scans were selected to provide a broad range of disease severity. The scoring system provided subscores for the presence and severity of 5 findings of CF lung disease. The sum of the subscores provided a total score. The CT scans were then read again by each of the radiologists at least 11 months later. Using Mixed Effects Linear Model Analysis, the sources of error (scan-to-scan variation, interrater variance, and intrarater variance) were calculated., Results: For the total score, the scan-to-scan variation was 14.48, interrater variance was 0.28, and intrarater variance was 0.45, with an overall reproducibility of 95%. The square root of scan-to-scan variance, a measure of sensitivity, was 3.81. Evaluation of the subscores showed higher reproducibility for bronchiectasis and hyperinflation (95% and 88%, respectively). The bronchiectasis score was more sensitive than the air-trapping score (1.46 vs. 0.89)., Discussion: This system was developed to provide a reproducible method that could be used to evaluate the lobar location, severity, and extent of a broad spectrum of CT features of CF lung disease, especially in children. This study demonstrates that the overall score is both sensitive to variation in the severity of lung disease and reproducible.