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Cystic fibrosis
Original article
Progression of early structural lung disease in young children with cystic fibrosis assessed using CT
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  1. Lauren S Mott1,2,
  2. Judy Park1,
  3. Conor P Murray3,
  4. Catherine L Gangell1,
  5. Nicholas H de Klerk1,
  6. Philip J Robinson4,5,6,
  7. Colin F Robertson4,5,6,
  8. Sarath C Ranganathan4,5,6,
  9. Peter D Sly1,7,
  10. Stephen M Stick1,2,8,
  11. on behalf of AREST CF1,4,5,8
  1. 1Telethon Institute for Child Health Research and Centre for Child Health Research, University of Western Australia, Perth, Australia
  2. 2School of Paediatrics and Child Health, University of Western Australia, Perth, Australia
  3. 3Department of Diagnostic Imaging, Princess Margaret Hospital for Children, Perth, Australia
  4. 4Department of Respiratory Medicine, Royal Children's Hospital, Melbourne, Australia
  5. 5Murdoch Children's Research Institute, Melbourne, Australia
  6. 6Department of Paediatrics, University of Melbourne, Melbourne, Australia
  7. 7Queensland Children's Medical Research Institute, University of Queensland, Brisbane, Australia
  8. 8Department of Respiratory Medicine, Princess Margaret Hospital for Children, Perth, Australia
  1. Correspondence to Professor Stephen M Stick, Department of Respiratory Medicine, Princess Margaret Hospital for Children, Subiaco, WA 6008, Australia; stephen.stick{at}health.wa.gov.au

Abstract

Background Cross-sectional studies implicate neutrophilic inflammation and pulmonary infection as risk factors for early structural lung disease in infants and young children with cystic fibrosis (CF). However, the longitudinal progression in a newborn screened population has not been investigated.

Aim To determine whether early CF structural lung disease persists and progresses over 1 year and to identify factors associated with radiological persistence and progression.

Methods 143 children aged 0.2–6.5 years with CF from a newborn screened population contributed 444 limited slice annual chest CT scans for analysis that were scored for bronchiectasis and air trapping and analysed as paired scans 1 year apart. Logistic and linear regression models, using generalised estimating equations to account for multiple measures, determined associations between persistence and progression over 1 year and age, sex, severe cystic fibrosis transmembrane regulator (CFTR) genotype, pancreatic sufficiency, current respiratory symptoms, and neutrophilic inflammation and infection measured by bronchoalveolar lavage.

Results Once detected, bronchiectasis persisted in 98/133 paired scans (74%) and air trapping in 178/220 (81%). The extent of bronchiectasis increased in 139/227 (63%) of paired scans and air trapping in 121/264 (47%). Radiological progression of bronchiectasis and air trapping was associated with severe CFTR genotype, worsening neutrophilic inflammation and pulmonary infection.

Discussion CT-detected structural lung disease identified in infants and young children with CF persists and progresses over 1 year in most cases, with deteriorating structural lung disease associated with worsening inflammation and pulmonary infection. Early intervention is required to prevent or arrest the progression of structural lung disease in young children with CF.

  • Cystic fibrosis
  • bronchiectasis
  • air trapping
  • CT
  • bronchoalveolar lavage
  • imaging/CT MRI etc
  • paediatric lung disease
  • asbestos induced lung disease
  • asthma epidemiology
  • clinical epidemiology
  • COPD epidemiology
  • interstitial fibrosis
  • lung cancer
  • mesothelioma
  • occupational lung disease
  • paediatric asthma
  • respiratory measurement
  • paediatric physician
  • lung physiology
  • airway epithelium
  • exhaled airway markers
  • sleep apnoea
  • tobacco and the lung

https://doi.org/10.1136/thoraxjnl-2011-200912

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    Footnotes

    • See Editorial, p 471

    • Funding AREST CF is funded by the Cystic Fibrosis Foundation Therapeutics, Inc (Sly040A, Stick090A), USA; the Australian Cystic Fibrosis Research Trust; and the National Health and Medical Research Council (513730), Australia. LSM is funded by a PhD scholarship from the National Health and Medical Research Council and supplementary scholarships from the University of Western Australia and the Australian Cystic Fibrosis Research Trust. This research was presented in part at the European Cystic Fibrosis Society Conference, European Respiratory Society Annual Scientific Meeting and Thoracic Society of Australia and New Zealand Annual Scientific Meeting, supported by the Lung Institute of Western Australia, the University of Western Australia and the Thoracic Society of Australia and New Zealand.

    • Competing interests None.

    • Ethics approval The study was approved by the ethics committees of the Princess Margaret Hospital, Perth, and Royal Children's Hospital, Melbourne.

    • Provenance and peer review Not commissioned; externally peer reviewed.

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