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Thorax 69:320-327 doi:10.1136/thoraxjnl-2013-204176
  • Cystic fibrosis
  • Original article

Is chest CT useful in newborn screened infants with cystic fibrosis at 1 year of age?

Open Access
  1. on behalf of the London Cystic Fibrosis Collaboration (LCFC)
  1. 1Portex Unit: Respiratory Physiology and Medicine, UCL Institute of Child Health, London, UK
  2. 2Department of Radiology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK
  3. 3Department of Paediatric Respiratory Medicine, Imperial College and Royal Brompton and Harefield Hospital NHS Foundation Trust, London, UK
  4. 4Respiratory Unit, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK
  5. 5Centre for Paediatric Epidemiology and Biostatistics, UCL Institute of Child Health, MRC Centre for Epidemiology of Child Health, London, UK
  6. 6Department of Anaesthesia, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK
  7. 7Department of Radiology, Cincinnati Children's Hospital, Cincinnati, Ohio, USA
  1. Correspondence to Dr Lena Priscilla Thia, Portex Respiratory Unit, UCL Institute of Child Health, 30 Guilford Street, London WC1N 1EH, UK; l.thia{at}ucl.ac.uk
  • Received 11 July 2013
  • Revised 20 September 2013
  • Accepted 26 September 2013
  • Published Online First 16 October 2013

Abstract

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.

This is an Open Access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 3.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/3.0/

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