The Journal of Pediatrics
Volume 155, Issue 5 , Pages 623-628.e1 , November 2009

Bronchiectasis in Infants and Preschool Children Diagnosed with Cystic Fibrosis after Newborn Screening

  • Stephen M. Stick, MA, MB BChir, PhD

      Affiliations

    • Princess Margaret Hospital for Children, University of Western Australia, Melbourne, Australia
    • TVW Institute for Child Health Research, University of Western Australia, Melbourne, Australia
    • School of Paediatrics and Child Health, University of Western Australia, Melbourne, Australia
    • Corresponding Author InformationReprint requests: Stephen Stick, Department of Respiratory Medicine, Princess Margaret Hospital for Children, Box D184, Perth 6010, Western Australia.
    • ,
  • Siobhain Brennan, BSc (hons), PhD

      Affiliations

    • TVW Institute for Child Health Research, University of Western Australia, Melbourne, Australia
    • ,
  • Conor Murray, MBBS, Dip Ch, FRANZCR

      Affiliations

    • Princess Margaret Hospital for Children, University of Western Australia, Melbourne, Australia
    • ,
  • Tonia Douglas, MBChB (hons), FRACP

      Affiliations

    • Princess Margaret Hospital for Children, University of Western Australia, Melbourne, Australia
    • TVW Institute for Child Health Research, University of Western Australia, Melbourne, Australia
    • ,
  • Britta S. von Ungern-Sternberg, MD, DEAA

      Affiliations

    • Princess Margaret Hospital for Children, University of Western Australia, Melbourne, Australia
    • ,
  • Luke W. Garratt, BSc (hons)

      Affiliations

    • TVW Institute for Child Health Research, University of Western Australia, Melbourne, Australia
    • ,
  • Catherine L. Gangell, BSc (hons), PhD

      Affiliations

    • TVW Institute for Child Health Research, University of Western Australia, Melbourne, Australia
    • ,
  • Nicholas De Klerk, BSc, MSc, F Ed TC, PhD

      Affiliations

    • TVW Institute for Child Health Research, University of Western Australia, Melbourne, Australia
    • ,
  • Barry Linnane, MD, PhD

      Affiliations

    • Perth and Royal Children's Hospital, Melbourne, Australia
    • ,
  • Sarath Ranganathan, MB ChB, MRCP, PhD, FRCPCH, FRACP

      Affiliations

    • Perth and Royal Children's Hospital, Melbourne, Australia
    • ,
  • Phillip Robinson, BMed Sc, MBBS, FRACP, MD, PhD

      Affiliations

    • Perth and Royal Children's Hospital, Melbourne, Australia
    • ,
  • Colin Robertson, MBBS, FRACP, MSc (Epi), MD

      Affiliations

    • Perth and Royal Children's Hospital, Melbourne, Australia
    • ,
  • Peter D. Sly, MBBS, FRACP, MD, DSci

      Affiliations

    • TVW Institute for Child Health Research, University of Western Australia, Melbourne, Australia
    • ,
  • Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST CF)

Received 29 October 2008 ,Revised 19 March 2009 ,Accepted 5 May 2009.

References 

  1. Cystic Fibrosis Foundation. Newborn screening. [Website]. Maryland: Cystic Fibrosis Foundation. [updated 2007 Feb 07; cited 2008 April 24]; Available from: http://www.cff.org/AboutCF/Testing/NewbornScreening/
  2. Stenbit A, Flume PA. Pulmonary complications in adult patients with cystic fibrosis. Am J Med Sci. 2008;335:55–59
  3. Twiss J, Stewart AW, Byrnes CA. Longitudinal pulmonary function of childhood bronchiectasis and comparison with cystic fibrosis. Thorax. 2006;61:414–418
  4. de Jong PA, Nakano Y, Hop WC, Long FR, Coxson HO, Pare PD, et al. Changes in airway dimensions on computed tomography scans of children with cystic fibrosis. Am J Respir Crit Care Med. 2005;172:218–224
  5. de Jong PA, Nakano Y, Lequin MH, Mayo JR, Woods R, Pare PD, et al. Progressive damage on high resolution computed tomography despite stable lung function in cystic fibrosis. Eur Respir J. 2004;23:93–97
  6. Massie J, Clements B. Diagnosis of cystic fibrosis after newborn screening: the Australasian experience—twenty years and five million babies later: a consensus statement from the Australasian Paediatric Respiratory Group. Pediatr Pulmonol. 2005;39:440–446
  7. Bell S, Robinson P. Cystic Fibrosis Standards of Care. North Ryde: Cystic Fibrosis Australia; 2008.
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  12. Brennan S, Hall GL, Horak F, Moeller A, Pitrez PM, Franzmann A, et al. Correlation of forced oscillation technique in preschool children with cystic fibrosis with pulmonary inflammation. Thorax. 2005;60:159–163
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  15. Davis SD, Fordham LA, Brody AS, Noah TL, Retsch-Bogart GZ, Qaqish BF, et al. Computed tomography reflects lower airway inflammation and tracks changes in early cystic fibrosis. Am J Respir Crit Care Med. 2007;175:943–950
  16. Long FR, Williams RS, Castile RG. Structural airway abnormalities in infants and young children with cystic fibrosis. J Pediatr. 2004;144:154–161
  17. Martinez TM, Llapur CJ, Williams TH, Coates C, Gunderman R, Cohen MD, et al. High-resolution computed tomography imaging of airway disease in infants with cystic fibrosis. Am J Respir Crit Care Med. 2005;172:1133–1138
  18. Australia CF. Patient Registry Report 2005. Sydney: Australian Cystic Fibrosis Association; 2007;
  19. Cystic Fibrosis Foundation. Patient Registry 2006 Annual Report. Bethesda: Cystic Fibrosis Foundation; 2008;
  20. Brennan S, Stick S, Douglas T, Murray C, Gangell C, Sturges N, et al. HRCT correlates with inflammation and symptoms in young children with cystic fibrosis. Respirology. 2007;12(Suppl):A22
  21. Gaillard EA, Carty H, Heaf D, Smyth RL. Reversible bronchial dilatation in children: comparison of serial high-resolution computer tomography scans of the lungs. Eur J Radiol. 2003;47:215–220
  22. Nixon GM, Armstrong DS, Carzino R, Carlin JB, Olinsky A, Robertson CF, et al. Early airway infection, inflammation, and lung function in cystic fibrosis. Arch Dis Child. 2002;87:306–311
  23. Brody AS, Kosorok MR, Li Z, Broderick LS, Foster JL, Laxova A, et al. Reproducibility of a scoring system for computed tomography scanning in cystic fibrosis. J Thorac Imaging. 2006;21:14–21
  24. de Jong PA, Ottink MD, Robben SG, Lequin MH, Hop WC, Hendriks JJ, et al. Pulmonary disease assessment in cystic fibrosis: comparison of CT scoring systems and value of bronchial and arterial dimension measurements. Radiology. 2004;231:434–439
  25. Helbich TH, Heinz-Peer G, Eichler I, Wunderbaldinger P, Gotz M, Wojnarowski C, et al. Cystic fibrosis: CT assessment of lung involvement in children and adults. Radiology. 1999;213:537–544
  26. Nathanson I, Conboy K, Murphy S, Afshani E, Kuhn JP. Ultrafast computerized tomography of the chest in cystic fibrosis: a new scoring system. Pediatr Pulmonol. 1991;11:81–86
  27. de Jong PA, Lindblad A, Rubin L, Hop WC, de Jongste JC, Brink M, et al. Progression of lung disease on computed tomography and pulmonary function tests in children and adults with cystic fibrosis. Thorax. 2006;61:80–85
  28. Bonnel AS, Song SM, Kesavarju K, Newaskar M, Paxton CJ, Bloch DA, et al. Quantitative air-trapping analysis in children with mild cystic fibrosis lung disease. Pediatr Pulmonol. 2004;38:396–405
  29. Puderbach M, Eichinger M, Haeselbarth J, Ley S, Kopp-Schneider A, Tuengerthal S, et al. Assessment of morphological MRI for pulmonary changes in cystic fibrosis CF) patients: comparison to thin-section CT and chest x-ray. Invest Radiol. 2007;42:715–725
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 This study is supported by Cystic Fibrosis Foundation (USA) (Project grant support), National Health and Medical Research Council (Australia) (Project grant support and Fellowships [S.M.S., P.D.S.]), and) Cystic Fibrosis (Australia) (Project grant support). The authors declare no conflicts of interest.

PII: S0022-3476(09)00474-0

doi: 10.1016/j.jpeds.2009.05.005

The Journal of Pediatrics
Volume 155, Issue 5 , Pages 623-628.e1 , November 2009

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