PT  - JOURNAL ARTICLE
AU  - Leonie Tepper
AU  - Elisabeth Utens
AU  - Alexandra Quittner
AU  - Karla Gonzalez-Graniel
AU  - Hugo Duivenvoorden
AU  - Harm Tiddens
TI  - The number of exacerbations in children and adolescents with cystic fibrosis (CF) can be predicted by computed tomography (CT) bronchiectasis (BE) score and quality of life scores
DP  - 2011 Sep 01
TA  - European Respiratory Journal
PG  - p905
VI  - 38
IP  - Suppl 55
4099  - http://erj.ersjournals.com/content/38/Suppl_55/p905.short
4100  - http://erj.ersjournals.com/content/38/Suppl_55/p905.full
SO  - Eur Respir J2011 Sep 01; 38
AB  - Background: CF is characterized by progressive BE and small airways disease. BE is related to the number of exacerbations and Health Related Quality of Life (HRQoL), assessed by the Cystic Fibrosis Questionnaire-Revised (CFQ-R).Objective: 1) Investigate predictive value of BE score and CFQ-R respiratory domain score (CFQ-R resp) for exacerbations. 2) Determine added predictive value of CFQ-R resp on top of BE score to predict number of exacerbations in the year following.Methods: Cohort study (July 2007 to Jan 2010) in clinical stable children and adolescents with CF, whom had routine chest CT and CFQ-R as part of their (bi-)annual examination. CT scans were anonymized and randomly scored using CFCT BE score, expressing BE as% of maximum score. CFQ-R was completed by children aged 6-13 years and their parents or by adolescents aged ≥ 14 years. Score-range 0-100, higher scores indicate better HRQoL. To compute predictive value of BE-score and CFQ-R resp the negative binomial regression was used and for added predictive value McFadden's R2.Results: CF patients (n=72); CFQ-Rs: child CFQ-Rs (n=40); parental CFQ-Rs (n=37); adolescent CFQ-Rs (n=32). Median CFQ-R resp score 83 (range 11-100) points. BE score positive predictive (p<0.01, rs=0.11), CFQ-R resp negative predictive (p<0.01, rs=-0.04) for number of exacerbations. Added predictive value of CFQ-R resp on top of BE score to predict number of exacerbations equalled 6%.Conclusion: BE and HRQoL have potentialities to predict the number of exacerbations in CF. A point increase on the CFQ-R resp results in a 6% reduction of the number of exacerbations.