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Oxygen therapy for cystic fibrosis

  1. Heather E Elphick1,*,
  2. George Mallory2

Editorial Group: Cochrane Cystic Fibrosis and Genetic Disorders Group

Published Online: 25 JUL 2013

Assessed as up-to-date: 12 AUG 2013

DOI: 10.1002/14651858.CD003884.pub4

How to Cite

Elphick HE, Mallory G. Oxygen therapy for cystic fibrosis. Cochrane Database of Systematic Reviews 2013, Issue 7. Art. No.: CD003884. DOI: 10.1002/14651858.CD003884.pub4.

Author Information

  1. 1

    Sheffield Children's Hospital, Respiratory Unit, Sheffield, UK

  2. 2

    Baylor College of Medicine, Texas Children's Hospital, Houston, Texas, USA

*Heather E Elphick, Respiratory Unit, Sheffield Children's Hospital, Western Bank, Sheffield, S10 2TH, UK. H.Elphick@sheffield.ac.uk. Heather.Elphick@sch.nhs.uk.

Publication History

  1. Publication Status: Stable (no update expected for reasons given in 'What's new')
  2. Published Online: 25 JUL 2013

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References

References to studies included in this review

  1. References to studies included in this review
  2. References to studies excluded from this review
  3. Additional references
  4. References to other published versions of this review
Barker 1998 {published data only}
  • Barker M, Wieching R, Hoffmann U, Ebfeld D, Kusenbach G. Effects of hyperoxia on oxygen uptake kinetics in cystic fibrosis patients and healthy controls [abstract]. European Respiratory Journal 1998;12(Suppl 28):391s.
Falk 2006 {published data only}
  • Falk B, Nini A, Zigel L, Yahav Y, Aviram M, Rivlin J, et al. Effect of low altitude at the Dead Sea on exercise capacity and cardiopulmonary response to exercise in cystic fibrosis patients with moderate to severe lung disease. Pediatric Pulmonology 2006;41(3):234-41.
Gozal 1997 {published data only}
Marcus 1992 {published data only}
  • Marcus CL, Bader D, Stabile MW, Wang C-I, Osher AB, Keens TG. Supplementation oxygen and exercise performance in patients with cystic fibrosis with severe pulmonary disease. Chest 1992;101(1):52-7.
McKone 2002 {published data only}
  • McKone EF, Barry S, Fitzgerald MX, Gallagher CG. The role of pulmonary mechanics and arterial hypoxaemia in exercise limitation in patients with severe cystic fibrosis [abstract]. American Journal of Respiratory and Critical Care Medicine 2000;161(3 Suppl):A228.
  • McKone EF, Barry SC, FitzGerald MX, Gallagher CG. The role of supplemental oxygen during submaximal exercise in patients with cystic fibrosis. European Respiratory Journal 2002;20(1):134-42.
  • McKone EF, Barry SC, Fitzgerald MX, Gallagher CG. Role of arterial hypoxemia and pulmonary mechanics in exercise limitation in adults with cystic fibrosis. Journal of Applied Physiology 2005;99(3):1012-8.
Milross 2001b {published data only}
  • Milross MA, Piper AJ, Norman M, Becker HF, Willson GN, Grunstein RR, et al. Low-flow oxygen and bilevel ventilatory support: effects on ventilation during sleep in cystic fibrosis. American Journal of Respiratory and Critical Care Medicine 2001;163(1):129-34.
  • Milross MA, Piper AJ, Norman M, Becker HF, Willson GN, Grunstein RR, et al. The effects of low flow oxygen and bilevel ventilatory support on gas exchange and ventilation during sleep in patients with cystic fibrosis [abstract]. Pediatric Pulmonology 1999;28 Suppl 19:285.
Nixon 1990 {published data only}
  • Nixon PA, Orenstein DM, Curtis SE, Ross EA. Oxygen supplementation during exercise in cystic fibrosis. American Review of Respiratory Disease 1990;142(4):807-11.
Parsons 1996 {published data only}
  • Parsons S, Grunstein R, Torzillo P, Sullivan C, Bye PTP. Nocturnal low-flow oxygen (nLFO2) improves oxyhaemoglobin saturation but not sleep function or carbon dioxide levels in patients with cystic fibrosis (CF) [abstract]. Australian & New Zealand Journal of Medicine 1997;27:256.
  • Parsons SW, Grunstein R, Sullivan CE, Bye PTP. The effects of nocturnal low-flow oxygen on sleep and gas exchange in patients with cystic fibrosis [abstract]. Pedriatric Pulmonology 1995;20 Suppl 12:251.
  • Parsons SW, Grunstein R, Torzillo PJ, Sullivan CE, Bye PTP. The effects of nocturnal low-flow oxygen (nLFO2) on sleep function and gas exchange in patients with cystic fibrosis (CF) [abstract]. American Journal of Respiratory and Critical Care Medicine 1996;153(Suppl):A72.
Shah 1997 {published data only}
  • Shah AR, Keens TG, Gozal D. Effect of supplemental oxygen on anaerobic exercise performance and recovery in normals and cystic fibrosis [abstract]. American Journal of Respiratory and Critical Care Medicine 1996;153(Suppl):A652.
  • Shah AR, Keens TG, Gozal D. Effect of supplemental oxygen on supramaximal exercise performance and recovery in cystic fibrosis. Journal of Applied Physiology 1997;83(5):1641-7.
Spier 1984 {published data only}
  • Spier S, Hughes D, Rivlin J, Levison H. Ventilatory and sleep responses to oxygen in cystic fibrosis patients [abstract]. 23rd Annual Meeting Cystic Fibrosis Club Abstracts; 1982 May 14; Washington D.C. 1982:65.
  • Spier S, Rivlin J, Hughes D, Levison H. The effect of oxygen on sleep, blood gases, and ventilation in cystic fibrosis. The American Review of Respiratory Disease 1984;129:712-8.
Zinman 1989 {published data only}
  • Zinman R, Corey M, Coates AL, Canny GJ, Connolly J, Levison H, et al. Nocturnal home oxygen in the treatment of hypoxemic cystic fibrosis patients. Journal of Pediatrics 1989;114(3):368-77.
  • Zinman R, Corey M, Coates AL, Canny GJ, Connolly J, Levison H, et al. Nocturnal oxygen therapy in the treatment of hypoxemic cystic fibrosis patients [abstract]. Pediatric Pulmonology 1987;3(Suppl 3.1.3):73-4.

References to studies excluded from this review

  1. References to studies included in this review
  2. References to studies excluded from this review
  3. Additional references
  4. References to other published versions of this review
Elkins 2004 {published data only}
  • Elkins MR, Eberl S, Alison J, Bye P. The effect of bi-level non-invasive ventilation on mucociliary clearance in subjects with cystic fibrosis [abstract]. Pediatric Pulmonology 2004;38 Suppl 27:315.
Fauroux 1999 {published data only}
  • Fauroux B, Boule M, Lofaso F, Zerah F, Clement A, Harf A, et al. Chest physiotherapy in cystic fibrosis: improved tolerance with nasal pressure support ventilation. Pediatrics 1999;103(3):E32.
Fauroux 2000 {published data only}
  • Fauroux B, Itti E, Pigeot J, Isabey D, Meignan M, Ferry G, et al. Optimization of aerosol deposition by pressure support in children with cystic fibrosis: an experimental and clinical study. American Journal of Respiratory and Critical Care Medicine 2000;162(6):2265-71.
Fauroux 2001 {published data only}
  • Fauroux B, Pigeot J, Polkey MI, Isabey D, Clement A, Lofaso F. In vivo physiologic comparison of two ventilators used for domiciliary ventilation in children with cystic fibrosis. Critical Care Medicine 2001;29(11):2097-105.
Fauroux 2004 {published data only}
  • Fauroux B, Louis B, Essouri S, Leroux K, Lofaso F. Back up rate during non invasive mechanical ventilation in cystic fibrosis [abstract]. European Respiratory Journal 2003;22(Suppl 45):262s.
  • Fauroux B, Louis B, Hart N, Essouri S, Leroux K, Clement A, et al. The effect of back-up rate during non-invasive ventilation in young patients with cystic fibrosis. Intensive Care Medicine 2004;30(4):673-81.
Holland 2003 {published data only}
  • Holland A, Denehy L, Ntoumenopoulos G, McMeeken J, Wilson J. Non-invasive ventilation prevents inspiratory muscle fatigue and oxygen desaturation during airway clearance in adults with acute exacerbations of cystic fibrosis [abstract]. Proceedings of the Thoracic Society of Australia & New Zealand Annual Scientific Meeting; 2003 April 4-9; Adelaide, Australia 2003. 2003:Abst #P140.
  • Holland A, Denehy L, Ntoumenopoulos G, Naughton M, Wilson J. Non-invasive ventilation prevents inspiratory muscle fatigue and oxygen desaturation during airway clearance in adults with acute exacerbations of cystic fibrosis [abstract]. Journal of Cystic Fibrosis 2003;2 Suppl 1:S62.
  • Holland A, Denehy L, Ntoumenopoulos G, Wilson J. Non-invasive ventilation prevents inspiratory muscle fatigue and oxygen desaturation during airway clearance in adults with exacerbations of cystic fibrosis [abstract]. American Journal of Respiratory and Critical Care Medicine 2003;167:D041.
  • Holland AE, Denehy L, Ntoumenopoulos G, Naughton MT, Wilson JW. Non-invasive ventilation assists chest physiotherapy in adults with acute exacerbations of cystic fibrosis. Thorax 2003;58(10):880-4.
Placidi 2006 {published data only}
  • Placidi G, Cornacchia M, Cappelletti LM, Mastella G, Assael BM, Braggion C. Short-term effects of positive airway pressure on sputum clearance by directed coughing: A cross-over, randomized study [abstract]. Pediatric Pulmonology 2001;32 Suppl 22:313-4.
  • Placidi G, Cornacchia M, Polese G, Zanolla L, Assael BM, Braggion C. Chest physiotherapy with positive airway pressure: a pilot study of short-term effects on sputum clearance in patients with cystic fibrosis and severe airway obstruction. Respiratory Care 2006;51(10):1145-53.
Riethmueller 2006 {published data only}
  • Riethmueller J, Borth-Bruhns T, Kumpf M, Vonthein R, Wiskirchen J, Stern M, et al. Recombinant human deoxyribonuclease shortens ventilation time in young, mechanically ventilated children. Pediatric Pulmonology 2006;41(1):61-6.
Serra 2002 {published data only}
  • Serra A, Appendini L, Braggion C, Mastella G, Rossi A, Polese G. Noninvasive proportional assist ventilation (PAV) and pressure support ventilation (PSV) in patients with cystic fibrosis (CF): an overnight study [abstract]. American Journal of Respiratory and Critical Care Medicine 2000;161(3 Suppl):A555.
  • Serra A, Polese G, Braggion C, Rossi A. Non-invasive proportional assist and pressure support ventilation in patients with cystic fibrosis and chronic respiratory failure. Thorax 2002;57(1):50-4.
Stewart 2001 {published data only}
  • Stewart IB, Sheel AW, Frangiolas DD, Wilcox PG, McKenzie DC. The effect of nitric oxide inhalation on pulmonary gas exchange and exercise capacity in patients with cystic fibrosis. Clinical Exercise Physiology 2001;3(1):44-8.
Young 2008 {published data only}
  • Young AC, Wilson JW, Kotsimbos TC, Naughton MT. Online Data Supplement to 'Randomised placebo controlled trial of non-invasive ventilation for hypercapnia in cystic fibrosis' [online]. Thorax 2008;63(1):72-7 online.
  • Young AC, Wilson JW, Kotsimbos TC, Naughton MT. Randomised placebo controlled trial of non-invasive ventilation for hypercapnia in cystic fibrosis. Thorax 2008;63(1):72-7.
  • Young AC, Wilson JW, Kotsimbos TC, Naughton MT. Randomized placebo-controlled trial of non-invasive ventilation for hypercapnia in cystic fibrosis [abstract]. Proceedings of Japanese Society of Sleep Research Conference. 2006:A9-A10.

Additional references

  1. References to studies included in this review
  2. References to studies excluded from this review
  3. Additional references
  4. References to other published versions of this review
ATS 1991
  • American Thoracic Society. Lung function testing: selection of reference values and interpretative strategies. American Review of Respiratory Disease 1991;144(5):1202-18.
Bradley 1999
Coffey 1991
Curtin 2002
Elbourne 2002
  • Elbourne DR, Altman DG, Higgins JPT, Curtin F, Worthington HV, Vail A. Meta-analysises involving cross-over trials: methodological issues. International Journal of Epidemiology 2002;31(1):140-9.
Fitzsimmons 1996
Frangiolis 2001
Fraser 1999
Frederiksen 1996
Gong 1992
Gorecka 1997
  • Gorecka D, Gorzelak K, Sliwinski P, Tobiasz M, Zielinski J. Effect of long-term oxygen therapy on survival in patients with chronic obstructive pulmonary disease with moderate hypoxaemia. Thorax 1997;52(8):674-9.
Higgins 2003
Higgins 2009
  • Higgins JPT, Altman DG. Chapter 8: Assessing risk of bias in included studies. In: Higgins JPT, Green S (editors). Cochrane Handbook of Systematic Reviews of Interventions. Version 5.0.2 [updated September 2009]. The Cochrane Collaboration, 2009. Available from www.cochrane-handbook.org. Cochrane Collaboration 2009.
Hodson 1991
  • Hodson ME, Madden BP, Steven MH, Tsang VT, Yacoub MH. Non-invasive mechanical ventilation for cystic fibrosis patients: a potential bridge to transplantation. European Respiratory Journal 1991;4(5):524-7.
Milross 2001a
Moran 2007
MRC 1981
  • Report of the Medical Research Council Working Party. Long term domiciliary oxygen therapy in chronic hypoxic cor pulmonale complicating chronic bronchitis and emphysema. The Lancet 1981;8222(1):681-6.
Narang 2003
NOTTG 1980
  • Nocturnal Oxygen Therapy Trial Group. Continuous or nocturnal oxygen therapy in hypoxemic chronic obstructive lung disease: a clinical trial. Annals of Internal Medicine 1980;93(3):391-8.
Ramsey 1996
  • Ramsey BW. Management of pulmonary disease in patients with cystic fibrosis. New England Journal of Medicine 1996;335(3):179-88.
Schidlow 1993
Tarpy 1995
Tepper 1983

References to other published versions of this review

  1. References to studies included in this review
  2. References to studies excluded from this review
  3. Additional references
  4. References to other published versions of this review
Elphick 2009
Elphick 2013
Mallory 2005
Mallory 2009
  • Mallory GB, Fullmer JJ, Vaughan DJ. Oxygen therapy for cystic fibrosis. Cochrane Database of Systematic Reviews 2009, Issue 1. [DOI: 10.1002/14651858.CD003884.pub2]