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London Cystic Fibrosis Collaboration Publications and Achievements

The collaboration has resulted in a large number of peer reviewed articles, international presentations and higher degrees.

The London Cystic Fibrosis Collaboration (LCFC) was established in 1998 by Professor Janet Stocks, Professor Carol Dezateux, Professor Andrew Bush and Professor John Price, and has been continuously supported by peer-reviewed funding. More information about the research cohorts can be found on the background page.

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Please see the links below for more details about the LCFC research output*

LCFC Main Achievements

  • The development and validation of sensitive, non-invasive methods of assessing lung function that can be used from birth
  • The development of reliable lung growth charts (reference data) to interpret lung function results from infancy through to adulthood
  • The implementation of standardised protocols across the LCFC sites for assessing lung structure (computed tomography (CT) scans) and early signs of infection and inflammation (bronchoalveolar lavage (BAL))
  • Longitudinal studies in children with CF and healthy controls, which enable measures of lung function, structure and inflammation to be related to clinical status from birth through to adulthood

lung function at different ages

Main Publications for LCFC Cohort 1

  • Owens CM, Aurora P, Stanojevic S, Bush A, Wade A, Oliver C, Calder A, Price J, Carr SB, Shankar A, Stocks J, on behalf of the London Collaborative Cystic Fibrosis S. Lung Clearance Index and HRCT are complementary markers of lung abnormalities in young children with CF. Thorax 2011; 66: 481-8.
  • Aurora P, Stanojevic S, Wade A, Oliver C, Kozlowska W, Lum S, Bush A, Price J, Carr SB, Shankar A, Stocks J. Lung Clearance Index at 4 years Predicts Subsequent Lung Function in Children with Cystic Fibrosis. Am J Respir Crit Care Med 2011; 183: 752-8.
  • Kozlowska W, Bush A, Wade A, Aurora P, Carr SB, Castle RA, Hoo AF, Lum S, Price J, Ranganathan S, Saunders C, Stanojevic S, Stroobant J, Wallis CE, Stocks J, on behalf of the London Collaborative Cystic Fibrosis S. Lung Function from Infancy to the Preschool Years After Clinical Diagnosis of Cystic Fibrosis. Am J Respir Crit Care Med 2008; 178: 42-9.
  • Lum S, Gustafsson P, Ljungberg H, Hulskamp G, Bush A, Carr SB, Castile R, Hoo AF, Price JF, Ranganathan SC, Stroobant J, Wade A, Wallis CE, Wyatt H, Stocks J, London Collaborative Cystic Fibrosis G. Early detection of cystic fibrosis lung disease: multiple-breath washout vs. raised volume tests. Thorax 2007; 62: 341-
  • Aurora P, Kozlowska WJ, Stocks J. Gas mixing efficiency from birth to adulthood measured by multiple-breath washout. Respir Physiol Neurobiol 2005; 148: 125-39.
  • Aurora P, Bush A, Gustafsson P, Oliver C, Wallis CE, Price J, Stroobant J, Carr SB, Stocks J, London Collaborative Cystic Fibrosis G. Multiple-breath washout as a marker of lung disease in preschool children with cystic fibrosis. Am J Respir Crit Care Med 2005; 179   249-56.
  • Ranganathan S, Stocks J, Dezateux C, Bush A, Wade A, Carr SB, Castle R, Dinwiddie RD, Hoo AF, Lum S, Price J, Stroobant J, Wallis CE, London Collaborative Cystic Fibrosis G. The evolution of airway function in infancy and early childhood following clinical diagnosis of cystic fibrosis. Am J Respir Crit Care Med 2004; 169: 928-33.
  • Kozlowska WJ, Aurora P, Stocks J. The use of computer-animation programs during spirometry in preschool children. Eur Respir J 2004; 23: 494-5.
  • Aurora P, Stocks J, Oliver C, Saunders C, Castle R, Chaziparasidis G, Bush A, on behalf of the London Collaborative Cystic Fibrosis S. Quality control for spirometry in preschool children with and without lung disease. Am J Respir Crit Care Med 2004; 169: 1152-9.
  • Aurora P, Gustafsson P, Bush A, Lindblad A, Oliver C, Wallis CE, Stocks J. Multiple-breath inert gas washout as a measure of ventilation distribution in children with cystic fibrosis. Thorax 2004; 59: 1068-73.
  • Ranganathan S, Goetz I, Hoo AF, Lum S, Castle R, Stocks J, London Collaborative Cystic Fibrosis G. Assessment of tidal breathing parameters in infants with cystic fibrosis. Eur Respir J 2003; 22: 761-6.
  • Ranganathan S, Bush A, Dezateux CA, Carr SB, Hoo AF, Lum S, Madge SL, Price J, Stroobant J, Wade A, Wallis CE, Wyatt H, Stocks J. Relative ability of full and partial forced expiratory maneuvers to identify diminished airway function in infants with cystic fibrosis. Am J Respir Crit Care Med 2002; 166: 1350-7.
  • Ranganathan S, Dezateux CA, Bush A, Carr SB, Castle R, Madge SL, Price JF, Stroobant J, Wade AM, Wallis CE, Stocks J. Airway function in infants newly diagnosed with cystic fibrosis. Lancet 2001; 358: 1964-5.

Main Publications for LCFC Cohort 2 

  • Davies G, Thia LP, Stocks J, Bush A, Hoo AF, Wade A, Nguyen TT, Brody S, Calder A, Klein, N, Carr SB, Wallis C, Suri R, Ruiz G, Balfour-Lynn I, on behalf of the London Cystic Fibrosis Collaboration (LCFC).Minimal change in structural, functional and inflammatory markers of lung disease in newborn screened infants with cystic fibrosis at one year Journal of Cystic Fibrosis, 2020 Feb in press doi: 10.1016/j.jcf.2020.01.006.
  •  Davies G, Stanojevic S, Raywood E, Duncan J, Stocks J, Lum S, Bush A, Viviani L, Wade A, Calder A, Owens CM, Goubau C, Carr SB, Bossley CJ, Pao C, Aurora A. An observational study of the lung clearance index throughout childhood in cystic fibrosis: Early years matter. Eur Respir J 2020; in press (https://doi.org/10.1183/13993003.00006-2020).
  • Davies G, Stocks J, Thia LP, Hoo A-F, Bush A, Aurora P, Brennan L, Lee S, Lum S, Cottam P, Miles J, Chudleigh J, Kirkby J, Balfour-Lynn IM, Carr SB, Wallis C, Wyatt H, Wade A, on behalf of the London Cystic Fibrosis Collaboration (LCFC). Pulmonary function deficits in newborn screened infants with cystic fibrosis managed with standard UK care are mild and transient. European Respiratory Journal. 2017, Nov 9;50(5). pii: 1700326.
  • Thia LP, Calder A, Stocks J, Bush A, Owens CM, Wallis C, Young C, Sullivan Y, Wade A, McEwan A, Brody AS, on behalf of the London Cystic Fibrosis C. Is chest CT useful in newborn screened infants with cystic fibrosis at 1 year of age? Thorax 2014; 69: 320-7.
  • Nguyen TT, Thia LP, Hoo AF, Bush A, Aurora P, Wade A, Chudleigh J, Lum S, Stocks J, on behalf of the London Cystic Fibrosis C. Evolution of lung function during the first year of life in newborn screened cystic fibrosis infants. Thorax 2014; 69: 910-7.
  • Chudleigh J, Hoo AF, Ahmed D, Prasad A, Sheehan D, Francis J, Buckingham S, Cowlard J, Thia L, Nguyen TT, Stocks J; London Cystic Fibrosis Collaboration (LCFC). Positive parental attitudes to participating in research involving newborn screened infants with CF. J Cyst Fibros. 2013 May;12(3):234-40. doi: 10.1016/j.jcf.2012.09.001. Epub 2012 Oct 5.
  • Nguyen TT, Hoo AF, Lum S, Wade A, Thia LP, Stocks J. New reference equations to improve interpretation of infant lung function. Pediatr Pulmonol. 2013 Apr;48(4):370-80. doi: 10.1002/ppul.22656. Epub 2012 Sep 4.
  • Hoo AF, Thia L, Nguyen TD, Bush A, Chudleigh J, Lum S, Ahmed D, Balfour-Lynn IM, Carr SB, Chavasse R, Costeloe K, Price J, Shankar A, Wallis C, Wyatt H, Stocks J, London Collaborative Cystic Fibrosis G. Lung function is abnormal in 3 month old infants with cystic fibrosis diagnosed by newborn screening. Thorax 2012; 67: 874-81.

Other collaborative publications

  • Verger N, Arigliani M, Raywood E, Duncan J, Negreskul Y, Bush A, Aurora A. Limitations of regional ventilation inhomogeneity indices in children with cystic fibrosis. Pediatric Pulmonology. 2020 May in press doi.org/10.1002/ppul.24863
  • Lum S, Bountziouka V, Wade A, Hoo AF, Kirkby J, Moreno-Galdo A, de Mir I, Sardon-Prado O, Corcuera-Elosegui P, Mattes J, Borrego LM, Davies G, Stocks J. New reference ranges for interpreting forced expiratory manoeuvres in infants and implications for clinical interpretation: a multicentre collaboration. Thorax. 2016 Mar;71(3):276-83. doi: 10.1136/thoraxjnl-2015-207278. Epub 2015 Nov 2.
  • Stanojevic S, Bilton D, McDonald A, Stocks J, Aurora P, Prasad A, Cole TJ, Davies G. Global Lung Function Initiative equations improve interpretation of FEV1 decline among patients with cystic fibrosis Eur Respir J. 2015 Jul;46(1):262-4. doi: 10.1183/09031936.00187314. Epub 2015 Apr 2. No abstract available.
  • Stanojevic S, Stocks J, Bountziouka V, Aurora P, Kirkby J, Bourke S, Carr SB, Gunn E, Prasad A, Rosenfeld M, Bilton D. The impact of switching to the new global lung function initiative equations on spirometry results in the UK CF registry.  J Cyst Fibros. 2014 May;13(3):319-27. doi: 10.1016/j.jcf.2013.11.006. Epub 2013 Dec 12. 
  • Lum S, Stocks J, Stanojevic S, Wade A, Robinson P, Gustafsson P, Brown M, Aurora P, Subbarao P, Hoo AF, Sonnappa S. Age and height dependence of lung clearance index and functional residual capacity.  Eur Respir J. 2013 Jun;41(6):1371-7. doi: 10.1183/09031936.00005512. Epub 2012 Nov 8.
  • Stocks J, Thia LP, Sonnappa S. Evaluation and use of childhood lung function tests in cystic fibrosis. Curr Opin Pulm Med. 2012 Nov;18(6):602-8. doi: 10.1097/MCP.0b013e328358dfbe. Review.
  • Lum S, Hoo AF, Hulskamp G, Wade A, Stocks J. Potential misinterpretation of infant lung function unless prospective healthy controls are studied.  Pediatr Pulmonol. 2010 Sep;45(9):906-13. doi: 10.1002/ppul.21255.
     

*Publication lists were last updated in July 2020, not all publications are listed. Analyses are still underway for the most recently collected data.

None of these achievements would have been possible without the incredible help of the children and their families who participated in the LCFC studies. Thank you.

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