title: Tracking CF disease progression with CT and respiratory symptoms in a cohort of children aged 6-19 years.
abstract: BACKGROUND
  Cystic fibrosis (CF) lung disease is characterized by bronchiectasis and trapped air on chest computed tomography (CT).
  
  
  OBJECTIVE
  We aim to validate bronchiectasis and trapped air as outcome measures by evaluating associations between changes in bronchiectasis, trapped air and patient-reported respiratory symptoms.
  
  
  METHODS
  A longitudinal cohort study has been conducted. CF patients (aged 6-19 years) who had two routine CTs and completed twice a Cystic Fibrosis Questionnaire-Revised within 2 years (referred to as T1 and T2 ), in the period of July 2007 to January 2012 were included. Bronchiectasis and trapped air were scored using the CF-CT scoring system. Correlation coefficients and student's paired t tests were performed.
  
  
  RESULTS
  In total 40 patients were included with a median age at T1 of 12.6 years (range 6-17 years), and at T2 14.5 years (range 8-19 years). At T1 , bronchiectasis (r = -0.49, P < 0.01) and trapped air (r = -0.34, P = 0.04) correlated with CFQ-R Respiratory Symptoms Scores (CFQ-R RSS). At T2 similar correlations were found with the CFQ-R RSS. Over 2 years, there was significant progression in bronchiectasis (P = 0.03) and trapped air (P = 0.03), but not in CFQ-R RSS. Changes in bronchiectasis and trapped air were not associated with changes in CFQ-R RSS.
  
  
  CONCLUSIONS
  Our results indicate that bronchiectasis and trapped are sensitive outcome measures in CF lung disease, showing a significant association with CFQ-R RSS at two-time points. However, progression of bronchiectasis and trapped air over 2 year does not necessarily correlate to changes in quality of life.
authors.affiliation: Department of Pediatric Pulmonology, Erasmus Medical Centre-Sophia Children's Hospital, Rotterdam, The Netherlands; Department of Radiology, Erasmus MC, Rotterdam, The Netherlands.
authors.forename: Leonie A
authors.initials: LA
authors.lastname: Tepper
authors.forename: Daan
authors.initials: D
authors.lastname: Caudri
authors.forename: Elisabeth M W J
authors.initials: EM
authors.lastname: Utens
authors.forename: Els C
authors.initials: EC
authors.lastname: van der Wiel
authors.forename: Alexandra L
authors.initials: AL
authors.lastname: Quittner
authors.forename: Harm A W M
authors.initials: HA
authors.lastname: Tiddens
mesh_terms: D000293:Adolescent; D000388:Air; D001987:Bronchiectasis / Q000000981:diagnostic imaging; D002648:Child; D015331:Cohort Studies; D003550:Cystic Fibrosis / Q000000981:diagnostic imaging / Q000453:epidemiology*; D018450:Disease Progression; D005260:Female; D006801:Humans; D008137:Longitudinal Studies; D008168:Lung / Q000000981:diagnostic imaging*; D008297:Male; D009426:Netherlands / Q000453:epidemiology; D011788:Quality of Life; D014057:Tomography, X-Ray Computed; D055815:Young Adult
keywords: imaging; outcome measures; quality of life; validation