title: Bronchiectases at early chest computed tomography in children with cystic fibrosis are associated with increased risk of subsequent pulmonary exacerbations and chronic pseudomonas infection.
abstract: BACKGROUND
  Children 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.
  
  
  OBJECTIVE
  1 - To assess the number of respiratory exacerbations (RTEs) during a follow-up period of 6 years 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.
  
  
  METHODS
  68 chest CT performed in patients without chronic PsA infection were scored. All patients (median age 7.8 years) had at least 4 clinical, functional and microbiologic assessments/year in the subsequent 6 years. RTE was defined as hospitalization and IV antibiotic treatment for respiratory symptoms.
  
  
  RESULTS
  86.8% patients had <3 RTEs in the 6 year follow-up period. The number of RTEs in the 6 years 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.
  
  
  CONCLUSIONS
  In CF children free from chronic PsA, both CT scores and FEV1 values demonstrate significant correlation with disease severity in the subsequent 6 years but CT score has higher predictive value in the identification of patients at risk.
authors.affiliation: Verona Regional Cystic Fibrosis Centre, Piazzale Stefani 1, 37126 Verona, Italy. Electronic address: carlafbortoluzzi@gmail.com.
authors.forename: Carla-Federica
authors.initials: CF
authors.lastname: Bortoluzzi
authors.affiliation: Verona Regional Cystic Fibrosis Centre, Piazzale Stefani 1, 37126 Verona, Italy.
authors.forename: Sonia
authors.initials: S
authors.lastname: Volpi
authors.affiliation: Verona Regional Cystic Fibrosis Centre, Piazzale Stefani 1, 37126 Verona, Italy.
authors.forename: Ciro
authors.initials: C
authors.lastname: D'Orazio
authors.affiliation: Departments of Pediatric Pulmonology and Radiology, Erasmus MC-Sophia Children's Hospital, Dr. Molewaterplein 60, 3015 GJ Rotterdam, The Netherlands; Department of Radiology, Erasmus MC-Sophia Children's Hospital, Dr. Molewaterplein 60, 3015 GJ Rotterdam, The Netherlands.
authors.forename: Harm A W M
authors.initials: HA
authors.lastname: Tiddens
authors.affiliation: Departments of Pediatric Pulmonology and Radiology, Erasmus MC-Sophia Children's Hospital, Dr. Molewaterplein 60, 3015 GJ Rotterdam, The Netherlands; Department of Radiology, Erasmus MC-Sophia Children's Hospital, Dr. Molewaterplein 60, 3015 GJ Rotterdam, The Netherlands.
authors.forename: Martine
authors.initials: M
authors.lastname: Loeve
authors.affiliation: Verona Regional Cystic Fibrosis Centre, Piazzale Stefani 1, 37126 Verona, Italy.
authors.forename: Gloria
authors.initials: G
authors.lastname: Tridello
authors.affiliation: Verona Regional Cystic Fibrosis Centre, Piazzale Stefani 1, 37126 Verona, Italy.
authors.forename: Baroukh M
authors.initials: BM
authors.lastname: Assael
mesh_terms: D002648:Child; D002675:Child, Preschool; D002908:Chronic Disease; D003550:Cystic Fibrosis / Q000150:complications* / Q000000981:diagnostic imaging*; D005260:Female; D005541:Forced Expiratory Volume; D006801:Humans; D008171:Lung Diseases / Q000000981:diagnostic imaging* / Q000382:microbiology / Q000503:physiopathology; D008297:Male; D015233:Models, Statistical; D011552:Pseudomonas Infections / Q000000981:diagnostic imaging* / Q000503:physiopathology; D011550:Pseudomonas aeruginosa; D013902:Radiography, Thoracic; D012720:Severity of Illness Index; D013147:Spirometry; D014057:Tomography, X-Ray Computed
keywords: Computed tomography; Cystic fibrosis; Pseudomonas aeruginosa; Respiratory exacerbations