PT  - JOURNAL ARTICLE
AU  - Lena P Thia
AU  - Alistair Calder
AU  - Janet Stocks
AU  - Andrew Bush
AU  - Catherine M Owens
AU  - Colin Wallis
AU  - Carolyn Young
AU  - Yvonne Sullivan
AU  - Angie Wade
AU  - Angus McEwan
AU  - Alan S Brody
AU  - on behalf of the London Cystic Fibrosis Collaboration (LCFC)
TI  - Is chest CT useful in newborn screened infants with cystic fibrosis at 1 year of age?
AID  - 10.1136/thoraxjnl-2013-204176
DP  - 2014 Apr 01
TA  - Thorax
PG  - 320--327
VI  - 69
IP  - 4
4099  - http://thorax.bmj.com/content/69/4/320.short
4100  - http://thorax.bmj.com/content/69/4/320.full
SO  - Thorax2014 Apr 01; 69
AB  - Rationale Sensitive outcome measures applicable in different centres to quantify and track early pulmonary abnormalities in infants with cystic fibrosis (CF) are needed both for clinical care and interventional trials. Chest CT has been advocated as such a measure yet there is no validated scoring system in infants. Objectives The objectives of this study were to standardise CT data collection across multiple sites; ascertain the incidence of bronchial dilatation and air trapping in newborn screened (NBS) infants with CF at 1 year; and assess the reproducibility of Brody-II, the most widely used scoring system in children with CF, during infancy. Methods A multicentre observational study of early pulmonary lung disease in NBS infants with CF at age 1 year using volume-controlled chest CT performed under general anaesthetic. Main results 65 infants with NBS-diagnosed CF had chest CT in three centres. Small insignificant variations in lung recruitment manoeuvres but significant centre differences in radiation exposures were found. Despite experienced scorers and prior training, with the exception of air trapping, inter- and intraobserver agreement on Brody-II score was poor to fair (eg, interobserver total score mean (95% CI) κ coefficient: 0.34 (0.20 to 0.49)). Only 7 (11%) infants had a total CT score ≥12 (ie, ≥5% maximum possible) by either scorer. Conclusions In NBS infants with CF, CT changes were very mild at 1 year, and assessment of air trapping was the only reproducible outcome. CT is thus of questionable value in infants of this age, unless an improved scoring system for use in mild CF disease can be developed.