Chest Computed Tomography in early and advanced cystic fibrosis lung disease: Optimizing protocols, image analysis and further validation

Cystic Fibrosis (CF) is one of the most common life-threatening autosomal recessive diseases in the Western World, with a reported incidence rate of approximately 1 in every 2.000 to 5.000 caucasian newborns in most European countries. The gene mutation that plays an important role in the pathoph

Bronchiectases at early chest computed tomography in children with cystic fibrosis are associated with increased risk of subsequent pulmonary exacerbations and chronic pseudomonas infection

AbstractBackgroundChildren with cystic fibrosis (CF) are often Pseudomonas aeruginosa (PsA) free and exhibit normal spirometry between the ages of 5 and 7. It is reported that computed tomography (CT) is more sensitive than FEV1 as an instrument in the identification of pulmonary disease. It is not known whether CF-CT scores in childhood may be used to highlight children at risk of developing severe disease.Aims1 — To assess the number of respiratory exacerbations (RTEs) during a follow-up period of 6years and their correlation with the CF-CT scores in young CF children. 2 — To assess whether PsA-negative CF children with high chest CF-CT scores are more likely to develop chronic PsA lung infection.Methods68 chest CT performed in patients without chronic PsA infection were scored. All patients (median age 7.8years) had at least 4 clinical, functional and microbiologic assessments/year in the subsequent 6years. RTE was defined as hospitalization and IV antibiotic treatment for respiratory symptoms.Results86.8% patients had <3 RTEs in the 6year follow-up period. The number of RTEs in the 6years subsequent to the CT scan was correlated to the bronchiectasis CT score (BCTS) (r=0.612; p<0.001) and to FEV1 at baseline (r=−0.495, p<0.001). A BCTS ≥17.5 identified patients with >3 RTEs during follow-up (sensitivity: 100%, specificity: 85%), while FEV1 did not. Only BCTS was significant in a logistic multivariate model (RR 1.15). BCTS was significantly lower and FEV1 higher in patients who did not develop chronic PsA infection by the end of the study.ConclusionIn CF children free from chronic PsA, both CT scores and FEV1 values demonstrate significant correlation with disease severity in the subsequent 6years but CT score has higher predictive value in the identification of patients at risk

Bronchiectasis and Pulmonary Exacerbations in Children and Young Adults With Cystic Fibrosis

Objective: Respiratory tract exacerbation rate (RTE-R) is a key clinical efficacy end point in cystic fibrosis (CF) trials. Chest CT scanning holds great potential as a surrogate end point. Evidence supporting the ability of CT scan scores to predict RTE-R is an important step in validating CT scanning as a surrogate end point. The objective of this study was to investigate the association between CT scan scores and RTE-R in a cohort of pediatric patients with CF. Methods: A retrospective review of data from pediatric patients with CF included chest CT scans, spirometry, and 2 years follow-up. RTE-R was defined as the number of IV antibiotics courses per year. CT scans were scored with the Brody-II system, assessing bronchiectasis, airway wall thickening, mucus, and opacities. Results: One hundred fifteen patients contributed 170 CT scans. Median age and FEV(1) at first CT scan were 12 years (range, 5-20 years) and 90% predicted (range, 23% predicted-132% predicted), respectively. Analyzing exacerbation counts using Poisson regression models, bronchiectasis score and FEV(1) both were found to be strong independent predictors of RTE-R in the subsequent 2 years. For the bronchiectasis score categorized in quartiles, RTE-R increased by factors of 1.8 (95% CI, 0.6-6.1; P = .31), 5.5 (95% CI, 1.9-15.4; P = .001), and 10.6 (95% CI, 3.8-29.4; P < .001), respectively, for each quartile compared with the quartile with the best (ie, lowest) scores. Similarly, time to first respiratory tract exacerbation was significantly associated with quartiles of both bronchiectasis score and FEV(1). Conclusions: The CT scan bronchiectasis score is strongly associated with RTE-R in pediatric patients with CF, providing an important piece of evidence in the validation of CT scans as an end point for CF clinical trials. CHEST 2011; 140(1):178-18