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National Guideline Alliance (UK). Cystic Fibrosis: Diagnosis and management. London: National Institute for Health and Care Excellence (NICE); 2017 Oct 25. (NICE Guideline, No. 78.)

Cover of Cystic Fibrosis

Cystic Fibrosis: Diagnosis and management.

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StudyReason for Exclusion
Andrews, J., Sathe, N. A., Krishnaswami, S., McPheeters, M. L., Nonpharmacologic airway clearance techniques in hospitalized patients: a systematic review, Respiratory Care, 58, 2160–86, 2013Cystic fibrosis not included.
App, E. M., Kieselmann, R., Reinhardt, D., Lindemann, H., Dasgupta, B., King, M., Brand, P., Sputum rheology changes in cystic fibrosis lung disease following two different types of physiotherapy: flutter vs autogenic drainage, Chest, 114, 171–7, 1998The comparison was not prioritised for inclusion in the protocol (flutter vs AD).
Arens, R., Gozal, D., Omlin, K. J., Vega, J., Boyd, K. P., Keens, T. G., Woo, M. S., Comparison of high frequency chest compression and conventional chest physiotherapy in hospitalized patients with cystic fibrosis, American Journal of Respiratory & Critical Care Medicine, 150, 1154–7, 1994In Cochrane SR Main (2005). The comparison (percussion postural drainage vs. high frequency oscillation) was not prioritised for inclusion in the protocol.
Barnes, F., Rowland, M., Watt, P., The high frequency chest compression (HFCC) vest as a complementary intervention in persons with cystic fibrosis who find it difficult to engage in effective exercise or physiotherapy, Irish Journal of Medical Science, 1), S517, 2011Abstract only.
Bauer, M. L., McDougal, J., Schoumacher, R. A., Comparison of manual and mechanical chest percussion in hospitalized patients with cystic fibrosis, Journal of Pediatrics, 124, 250–4, 1994In Cochrane SR Main (2005). It compares manual vs mechanical chest percussion. This comparison was not included in the protocol.
Boyd, S., Brooks, D., Agnew-Coughlin, J., Ashwell, J., Evaluation of the literature on the effectiveness of physical therapy modalities in the management of children with cystic fibrosis, Pediatric Physical Therapy, 6, 70–74, 1994Low quality systematic review (2/11 total score in AMSTAR checklist). Included studies were checked for eligibility.
Bradley, J. M., Moran, F. M., Elborn, J. S., Evidence for physical therapies (airway clearance and physical training) in cystic fibrosis: an overview of five Cochrane systematic reviews, Respiratory Medicine, 100, 191–201, 2006Provides an overview of Cochrane reviews. References were checked for relevance.
Button, B. M., Heine, R. G., Catto-Smith, A. G., Olinsky, A., Phelan, P. D., Ditchfield, M. R., Story, I., Chest physiotherapy in infants with cystic fibrosis: to tip or not? A five-year study, Pediatric Pulmonology, 35, 208–13, 2003In Cochrane SR Freitas (2015). The intervention (postural drainage) was not included in the protocol.
Button, B. M., Heine, R., Catto-Smith, A., Olinsky, A., Phelan, P. D., Story, I., A twelve month comparison of standard versus modified chest physiotherapy in twenty infants with cystic fibrosis [abstract], 11th Annual North American Cystic Fibrosis Conference. 299p., 1997Abstract only.
Button, B. M., Wilson, C., Dentice, R., Cox, N. S., Middleton, A., Tannenbaum, E., Bishop, J., Cobb, R., Burton, K., Wood, M., Moran, F., Black, R., Bowen, S., Day, R., Depiazzi, J., Doiron, K., Doumit, M., Dwyer, T., Elliot, A., Fuller, L., Hall, K., Hutchins, M., Kerr, M., Lee, A. L., Mans, C., O’Connor, L., Steward, R., Potter, A., Rasekaba, T., Scoones, R., Tarrant, B., Ward, N., West, S., White, D., Wilson, L., Wood, J., Holland, A. E. The Thoracic Society of Australia, New Zealand Leaders in Lung, Health, Physiotherapy for cystic fibrosis in Australia and New Zealand, 2016Clinical practice guideline.
Button, B., Herbert, R., Maher, C., Positive expiratory pressure therapy better maintains pulmonary function than postural drainage and percussion in patients with cystic fibrosis, Australian Journal of Physiotherapy, 44, 285–286, 1998Abstract only.
Button, B.M., Heine, R.G., Catto-Smith, A.G., Phelan, P.D., Olinsky, A., Chest physiotherapy, gastro-oesophageal reflux, and arousal in infants with cystic fibrosis, Archives of Disease in Childhood, 89, 435–439, 2004In Cochrane SR Freitas (2015). The intervention (postural drainage) was not included in the protocol.
Cantin, A. M., Bacon, M., Berthiaume, Y., Mechanical airway clearance using the frequencer electro-acoustical transducer in cystic fibrosis, Clinical & Investigative Medicine - Medecine Clinique et Experimentale, 29, 159–65, 2006Intervention not relevant to the protocol (frequencer).
Cappelletti, L. M., Cornacchia, M., Braggion, C., Zanolla, L., Mastella, G., Short-term effects of three chest physiotherapy regimens on patients with cystic fibrosis hospitalized for pulmonary exacerbation: a crossover randomized study, Excerpta Medica International Congress Series, 1034, 239–46, 1993Intervention not relevant to the protocol (postural drainage).
Cardoso, R. M. T., Viana, R. A., Physiotherapy intervention in Cystic Fibrosis: A systematic review, Arquivos de Medicina, 25, 186–195, 2011Not in English.
Carr, J., Pryor, J. A., Hodson, M. E., Self chest clapping: patients’ views and the effects on oxygen saturation, Physiotherapy, 81, 753–757, 1995Not RCT.
Centre for Reviews and Dissemination, Chest physical therapy management of patients with cystic fibrosis: a meta-analysis (Structured abstract), Database of Abstracts of Reviews of Effects, 2015Abstract only. Full paper assessed.
Cerny, F. J., Relative effects of bronchial drainage and exercise for in-hospital care of patients with cystic fibrosis, Physical Therapy, 69, 633–9, 1989In Cochrane SR Main (2005). Comparisons are bronchial drainage and exercise. Study not included as exercise is covered in another evidence review.
Davies, G. A., Banks, A. E., Agent, P., Osman, L. P., Bilton, D., Hodson, M. E., The use of high frequency chest wall oscillation during an acute infective pulmonary exacerbation of cystic fibrosis, Pediatric Pulmonology, 47, 366, 2012Abstract only.
Dentice, R. L., Elkins, M. R., Bye, P. T., Adults with cystic fibrosis prefer hypertonic saline before or during airway clearance techniques: a randomised crossover trial, Journal of Physiotherapy, 58, 33–40, 2012Intervention not included in protocol (hypertonic saline).
Dentice, R., Elkins, M. R., Bye, P. T., A randomised trial of the effect of timing of hypertonic saline inhalation in relation to airway clearance physiotherapy in adults with cystic fibrosis, Pediatric Pulmonology, 45, 384, 2010Abstract only.
Dunn, C. E., Davies, Z., Yacob, A. A., Everson, C. L., Milla, C. E., A comparison of the acute effects of two hand-held percussive devices, the electroflo 5000 and the G5 flimm-fighter, for airway clearance in patients with cystic fibrosis (CF), Pediatric Pulmonology, 49, 372, 2014Conference abstract.
Dunn, C., Davies, Z., Everson, C., Zirbes, J., Kim, L., Milla, C., Study of acute effects on pulmonary function and sputum production with high frequency chest oscillation (HFCWO) and postural drainage aided by handheld percussion (PD-HP), Pediatric Pulmonology, 48, 359, 2013Abstract only.
Dwyer, T. J., Cobb, R., Hall, K., Robbins, L., Kelly, P., Bell, S., Bye, P. T., Randomised controlled two-centre trial of non-invasive ventilation (NIV)-assisted chest physiotherapy (CPT) during an acute exacerbation of cystic fibrosis, Journal of Cystic Fibrosis, 9, S73, 2010Abstract only.
Dwyer, T. J., Robbins, L., Kelly, P., Piper, A. J., Bell, S. C., Bye, P. T., Non-invasive ventilation used as an adjunct to airway clearance treatments improves lung function during an acute exacerbation of cystic fibrosis: a randomised trial, Journal of Physiotherapy, 61, 142–7, 2015Intervention not prioritised for inclusion in the protocol (NIV as adjunct to airway clearance treatments).
Eaton, T., Young, P., Zeng, I., Kolbe, J., A randomized evaluation of the acute efficacy, acceptability and tolerability of flutter and active cycle of breathing with and without postural drainage in non-cystic fibrosis bronchiectasis, Chronic Respiratory Disease, 4, 23–30, 2007Cystic fibrosis not included.
Enright, S., Chatham, K., Ionescu, A. A., Unnithan, V. B., Shale, D. J., Inspiratory muscle training improves lung function and exercise capacity in adults with cystic fibrosis, Chest, 126, 405–11, 2004Intervention (inspiratory muscle training) not included in protocol.
Fainardi, V., Longo, F., Faverzani, S., Tripodi, M. C., Chetta, A., Pisi, G., Short-term effects of high-frequency chest compression and positive expiratory pressure in patients with cystic fibrosis, Journal of Clinical Medicine Research, 3, 279–84, 2011This study was included in the Cochrane SR McIlwaine (2015). Data incomplete: “the Fainardi study was a cross-over study from which data from the end of the first randomization arm could not be obtained (quote from Cochrane review p.16).
Falk, M., Kelstrup, M., Andersen, J. B., Kinoshita, T., Falk, P., Stovring, S., Gothgen, I., Improving the ketchup bottle method with positive expiratory pressure, PEP, in cystic fibrosis, European Journal of Respiratory Diseases, 65, 423–32, 1984Intervention (PEP FET) not included in review.
Fauroux, B., Boule, M., Lofaso, F., Zerah, F., Clement, A., Harf, A., Isabey, D., Chest physiotherapy in cystic fibrosis: improved tolerance with nasal pressure support ventilation, Pediatrics, 103, E32, 1999This study was included in the Cochrane SR Moran 2013. Inspiratory pressure support ventilation (PSV) not included in protocol.
Fauroux, B., Nicot, F., Essouri, S., Hart, N., Clement, A., Polkey, M. I., Lofaso, F., Setting of noninvasive pressure support in young patients with cystic fibrosis, European Respiratory Journal, 24, 624–30, 2004Intervention (invasive ventilation) not included in protocol, n = 10.
Flume, P. A., Robinson, K. A., O’Sullivan, B. P., Finder, J. D., Vender, R. L., Willey-Courand, D. B., White, T. B., Marshall, B. C., Clinical Practice Guidelines for Pulmonary Therapies, Committee, Cystic fibrosis pulmonary guidelines: airway clearance therapies, Respiratory Care, 54, 522–37, 2009Review. Individual studies were checked for relevance.
Freitas, Diana A., Dias, A. L. Fernando, Chaves, S. S. Gabriela, Ferreira, M. H. Gardenia, Ribeiro, T. D. Cibele, Guerra, Ricardo O., Mendonca, M. P. P. Karla, Standard (head-down tilt) versus modified (without head-down tilt) postural drainage in infants and young children with cystic fibrosis, Cochrane Database of Systematic Reviews, 2015Intervention (postural drainage) not included in the protocol.
Giles, D. R., Wagener, J. S., Accurso, F. J., Butler-Simon, N., Short-term effects of postural drainage with clapping vs autogenic drainage on oxygen saturation and sputum recovery in patients with cystic fibrosis, Chest, 108, 952–4, 1995Intervention (postural drainage) not included in the protocol.
Gondor, M., Nixon, P. A., Mutich, R., Rebovich, P., Orenstein, D. M., Comparison of Flutter device and chest physical therapy in the treatment of cystic fibrosis pulmonary exacerbation, Pediatric Pulmonology, 28, 255–60, 1999Comparison (PD percussion or vibration vs. PEP) not included in the protocol.
Gozal, D., Nocturnal ventilatory support in patients with cystic fibrosis: comparison with supplemental oxygen, The European Respiratory Journal 1997 Sep;10(9):1999–2003, 1997This study was included in the Cochrane SR Moran 2013. It looks at the comparison NIV vs control, but for 1 night only (8hr), no follow-up or repeat tests. N < 10 participants.
Guimaraes, F. S., Lopes, A. J., Moco, V. J., Cavalcanti de Souza, F., Silveira de Menezes, S. L., Eltgol acutelly improves airway clearance and reduces static pulmonary volumes in adult cystic fibrosis patients, Journal of Physical Therapy Science, 26, 813–6, 2014Intervention (ELTGOL) not included in the protocol.
Hofmeyr, J. L., Webber, B. A., Hodson, M. E., Evaluation of positive expiratory pressure as an adjunct to chest physiotherapy in the treatment of cystic fibrosis, Thorax, 41, 951–4, 1986This study was included in the Cochrane SR Mckoy 2012. The comparison PEP vs FET was not included in the protocol.
Holland, A. E., Denehy, L., Ntoumenopoulos, G., Naughton, M. T., Wilson, J. W., Non-invasive ventilation assists chest physiotherapy in adults with acute exacerbations of cystic fibrosis, Thorax, 58, 880–4, 2003This study was included in the Cochrane SR Moran 2013. The comparison ACBT vs ACBT and NIV not included in protocol.
Homnick, D. N., White, F., de Castro, C., Comparison of effects of an intrapulmonary percussive ventilator to standard aerosol and chest physiotherapy in treatment of cystic fibrosis, Pediatric Pulmonology, 20, 50–5, 1995Comparison (intrapulmonary percussive ventilator) not included in the protocol.
Hough, Judith L., Flenady, Vicki, Johnston, Leanne, Woodgate, Paul G., Chest physiotherapy for reducing respiratory morbidity in infants requiring ventilatory support, Cochrane Database of Systematic Reviews, -, 2008Cystic fibrosis not included.
Houston, B. W., Mills, N., Solis-Moya, A., Inspiratory muscle training for cystic fibrosis, Cochrane Database of Systematic Reviews, 11, CD006112, 2013Intervention (inspiratory muscle training) not included in the protocol.
Jarad, N. A., Powell, T., Smith, E., Evaluation of a novel sputum clearance technique--hydro-acoustic therapy (HAT) in adult patients with cystic fibrosis: a feasibility study, Chronic Respiratory Disease, 7, 217–27, 2010This study was included in the Cochrane SR Warnock 2013. The intervention (hydro-acoustic therapy) was not included in the protocol.
Kluft, J., Beker, L., Castagnino, M., Gaiser, J., Chaney, H., Fink, R. J., A comparison of bronchial drainage treatments in cystic fibrosis, Pediatric Pulmonology, 22, 271–4, 1996The comparison (chest physical therapy PD vs. high frequency oscillation) not relevant, as PD was not in the protocol.
Kofler, A.M., Carlesi, A., Cutrera, R., Leone, P., Lucidi, V., Rosati, S., Turchetta, A., Vezzoli, P., BiPAP versus PEP as chest physiotherapy in patients with cystic fibrosis [abstract], Pediatric Pulmonology, 26, 344, 1998-, 1998This study was included in the Cochrane SR Moran 2013. The comparison PEP vs NIV comparison was not in included in the protocol.
Konstan, M. W., Stern, R. C., Doershuk, C. F., Efficacy of the Flutter device for airway mucus clearance in patients with cystic fibrosis, Journal of Pediatrics, 124, 689–93, 1994The intervention was not included in the protocol (postural drainage).
Krasovskij, S., Amelina, E., Usacheva, M., Samoylenko, V., Krilova, N., High frequency chest wall oscillation (HFCWO) in the treatment of acute pulmonary exacerbation in adult cystic fibrosis (CF) patients, European Respiratory Journal, 42, 2013Abstract only.
Kriemler, S., Radtke, T., Christen, G., Kerstan-Huber, M., Hebestreit, H., Short-Term Effect of Different Physical Exercises and Physiotherapy Combinations on Sputum Expectoration, Oxygen Saturation, and Lung Function in Young Patients with Cystic Fibrosis, LungLung, 194, 659–64, 2016The intervention was not relevant to the protocol (combination of physiotherapy and exercise).
Lagerkvist, A. L., Sten, G. M., Redfors, S. B., Lindblad, A. G., Hjalmarson, O., Immediate changes in blood-gas tensions during chest physiotherapy with positive expiratory pressure and oscillating positive expiratory pressure in patients with cystic fibrosis, Respiratory Care, 51, 1154–61, 2006In Cochrane SR McIlwaine 2015. The outcome (oxygen tension) not relevant to the protocol.
Lima, C. A., De Andrade, A. D. F. D., Campos, S. L., Brandao, D. C., Fregonezi, G., Mourato, I. P., Aliverti, A., De Britto, M. C. A., Effects of noninvasive ventilation on treadmill 6-min walk distance and regional chest wall volumes in cystic fibrosis: Randomized controlled trial, Respiratory Medicine, 108, 1460–1468, 2014No comparison for NIV.
Maayan, C., Bar-Yishay, E., Yaacobi, T., Marcus, Y., Katznelson, D., Yahav, Y., Godfrey, S., Immediate effect of various treatments on lung function in infants with cystic fibrosis, Respiration, 55, 144–51, 1989The comparisons (n-acetyl cysteine, salbutamol) are not relevant to the protocol.
Main, Eleanor, Prasad, Ammani, van der Schans, Cees P., Conventional chest physiotherapy compared to other airway clearance techniques for cystic fibrosis, Cochrane Database of Systematic Reviews, -, 2013Cochrane review. Does not include comparisons relevant for to the protocol. Individual studies were checked for relevance.
McCarren, B., Alison, J. A., Physiological effects of vibration in subjects with cystic fibrosis, European Respiratory Journal, 27, 1204–9, 2006Outcomes not included in the protocol.
McIlwaine, P. M., Wong, L. T., Peacock, D., Davidson, A. G., Long-term comparative trial of conventional postural drainage and percussion versus positive expiratory pressure physiotherapy in the treatment of cystic fibrosis, Journal of Pediatrics, 131, 570–4, 1997In Cochrane SR Main 2005. Study not included as intervention (postural drainage) was not in the protocol.
McKoy, N. A., Saldanha, I. J., Odelola, O. A., Robinson, K. A., Active cycle of breathing technique for cystic fibrosis, Cochrane Database of Systematic Reviews, 12, CD007862, 2012Cochrane review. Does not include comparisons relevant to the protocol. Individual studies were checked for relevance.
McKoy, N. A., Wilson, L. M., Saldanha, I. J., Odelola, O. A., Robinson, K. A., Active cycle of breathing technique for cystic fibrosis, Cochrane Database of Systematic Reviews, 7, CD007862, 2016This Cochrane SR is an update to a previous SR (McKoy 2010) and was identified at reruns. No additional studies were included in this review.
Miller, S., Hall, D. O., Clayton, C. B., Nelson, R., Chest physiotherapy in cystic fibrosis: a comparative study of autogenic drainage and the active cycle of breathing techniques with postural drainage, Thorax, 50, 165–9, 1995In Cochrane SR McKoy 2012 and Robinson 2010. The intervention (combination of ACBT and postural drainage) was not prioritised for inclusion in the protocol.
Milne, S. M., Eales, C. J., A pilot study comparing two physiotherapy techniques in patients with cystic fibrosis, South African Journal of Physiotherapy, 60, 3, 2004Below sample size requirement (n > 10).
Modi, A. C., Cassedy, A. E., Quittner, A. L., Accurso, F., Sontag, M., Koenig, J. M., Ittenbach, R. F., Trajectories of adherence to airway clearance therapy for patients with cystic fibrosis, Journal of Pediatric Psychology, 35, 1028–37, 2010In Cochrane SR Morrison 2014. The comparison HFCWO vs postural drainage and percussion was not prioritised for inclusion in the protocol.
Morgan, K., Osterling, K., Gilbert, R., Dechman, G., Effects of Autogenic Drainage on Sputum Recovery and Pulmonary Function in People with Cystic Fibrosis: A Systematic Review, Physiotherapy CanadaPhysiother Can, 67, 319–26, 2015Moderate quality systematic review (AMSTAR score 6/11). All included studies checked for inclusion.
Mortensen, J., Falk, M., Groth, S., Jensen, C., The effects of postural drainage and positive expiratory pressure physiotherapy on tracheobronchial clearance in cystic fibrosis, Chest, 100, 1350–7, 1991In Cochrane SR Warnock 2013. Study not analysed in Cochrane review and N = 10 or less not included in review.
Newhouse, P.A., White, F., Marks, J.H., Homnick, D.N., The intrapulmonary percussive ventilator and flutter device compared to standard chest physiotherapy in patients with cystic fibrosis, Clinical Pediatrics, 37, 427–432, 1998Sample size not > 10 (n = 10).
Orlik, T., Evaluation of the efficiency of selected thoracic physiotherapy methods used in the treatment of patients with cystic fibrosis, Medycyna Wieku Rozwojowego, 4, 233–46, 2000Not in English.
Orlik, T., [Evaluation of autodrainage methods in a selected group of cystic fibrosis patients with home environment factors taken into consideration], Medycyna Wieku Rozwojowego, 4, 247–59, 2000Not English.
Orlik, T., Sands, D., Long-term evaluation of effectiveness for selected chest physiotherapy methods used in the treatment of cystic fibrosis, Medycyna Wieku Rozwojowego, 5, 245–57, 2001Not in English.
Osman, L. P., Roughton, M., Hodson, M. E., Pryor, J. A., Short-term comparative study of high frequency chest wall oscillation and European airway clearance techniques in patients with cystic fibrosis, Thorax, 65, 196–200, 2010In Cochrane SR Morrison 2014. The comparison ACBT manual physiotherapy vs. AD not prioritised for inclusion in the protocol.
Pfleger, A., Theissl, B., Oberwaldner, B., Zach, M. S., Self-administered chest physiotherapy in cystic fibrosis: a comparative study of highpressure PEP and autogenic drainage, Lung, 170, 323–30, 1992No follow up period.
Pryor, J. A., Tannenbaum, E., Scott, S. F., Burgess, J., Cramer, D., Gyi, K., Hodson, M. E., Beyond postural drainage and percussion: Airway clearance in people with cystic fibrosis, Journal of Cystic Fibrosis, 9, 187–92, 2010In Cochrane SR McIlwaine 2015. Data could not be analysed as only p values are provided for the quality of life outcome. Tool for measuring quality of life not included in the protocol.
Pryor, J. A., Webber, B. A., Hodson, M. E., Warner, J. O., The Flutter VRP1 as an adjunct to chest physiotherapy in cystic fibrosis, Respiratory Medicine, 88, 677–81, 1994The comparison (ACBT vs Flutter) was not prioritised for inclusion in the protocol.
Regelmann, W. E., Elliott, G. R., Warwick, W. J., Clawson, C. C., Reduction of sputum Pseudomonas aeruginosa density by antibiotics improves lung function in cystic fibrosis more than do bronchodilators and chest physiotherapy alone, American Review of Respiratory Disease, 141, 914–21, 1990The comparison (vs antibiotics) was not included the protocol.
Reisman, J. J., Rivington-Law, B., Corey, M., Marcotte, J., Wannamaker, E., Harcourt, D., Levison, H., Role of conventional physiotherapy in cystic fibrosis, Journal of Pediatrics, 113, 632–6, 1988The intervention (postural drainage) was not included in the protocol.
Robinson, K. A., McKoy, N., Saldanha, I., Odelola, O. A., Active cycle of breathing technique for cystic fibrosis, Cochrane Database of Systematic Reviews, CD007862, 2010Cochrane SR. Includes one comparison of interest, but relevant study (Miller 2005) has been excluded from this review as it compares ACBT with postural drainage, which is a comparison not prioritised for inclusion in the protocol.
Rodriguez Hortal, M. C., Nygren-Bonnier, M., Hjelte, L., Non-invasive ventilation as airway clearance technique in cystic fibrosis [with consumer summary], Physiotherapy Research International 2016 Feb 29:Epub ahead of print, 2016This comparison was not prioritised for inclusion in the protocol (NIV vs PEP).
Rossman, C. M., Waldes, R., Sampson, D., Newhouse, M. T., Effect of chest physiotherapy on the removal of mucus in patients with cystic fibrosis, American Review of Respiratory Disease, 126, 131–5, 1982In Cochrane SR Warnock 2013. Intervention (postural drainage) not included in the protocol.
Sanchez Riera, H., Dapena Fernandez, F. J., Gomez Dominguez, F., Ortega Ruiz, F., Elias Hernandez, T., Montemayor Rubio, T., Castillo Gomez, J., [Comparative study of the efficacy of 2 respiratory physiotherapy protocols for patients with cystic fibrosis], Archivos de bronconeumologia, 35, 275–9, 1999Not English.
Sandsund, C. A., Roughton, M., Hodson, M. E., Pryor, J. A., Musculoskeletal techniques for clinically stable adults with cystic fibrosis: a preliminary randomised controlled trial, Physiotherapy, 97, 209–17, 2011The comparison (musculoskeletal treatment) was not included in the protocol.
Santana-Sosa, E., Gonzalez-Saiz, L., Groeneveld, I. F., Villa-Asensi, J. R., Gomez de Aguero, M. I. B., Fleck, S. J., Lopez-Mojares, L. M., Perez, M., Lucia, A., Benefits of combining inspiratory muscle with ‘whole muscle’ training in children with cystic fibrosis: a randomised controlled trial [with consumer summary], British Journal of Sports Medicine 2013 Oct;48(20):1513–1517, 2013The intervention (inspiratory muscle training) was not included in the protocol.
Scherer, T. A., Barandun, J., Martinez, E., Wanner, A., Rubin, E. M., Effect of highfrequency oral airway and chest wall oscillation and conventional chest physical therapy on expectoration in patients with stable cystic fibrosis, Chest, 113, 1019–27, 1998No relevant numerical evidence.
Sokol, Gil, Vilozni, Daphna, Hakimi, Ran, Lavie, Moran, Sarouk, Ifat, Bar, Bat-El, Dagan, Adi, Ofek, Miryam, Efrati, Ori, The Short-Term Effect of Breathing Tasks Via an Incentive Spirometer on Lung Function Compared With Autogenic Drainage in Subjects With Cystic Fibrosis, Respiratory CareRespir Care, 60, 1819–1825, 2015Not an RCT.
Sontag, M. K., Quittner, A. L., Modi, A. C., Koenig, J. M., Giles, D., Oermann, C. M., Konstan, M. W., Castile, R., Accurso, F. J., Investigators,, Coordinators of the Airway Secretion Clearance, Trial, Lessons learned from a randomized trial of airway secretion clearance techniques in cystic fibrosis, Pediatric Pulmonology, 45, 291–300, 2010Incomplete reporting: only p values reported.
Steen, H. J., Redmond, A. O., O’Neill, D., Beattie, F., Evaluation of the PEP mask in cystic fibrosis, Acta Paediatrica Scandinavica, 80, 51–6, 1991The comparison (PEP vs PEP FET) was not prioritised for inclusion in the protocol.
Swaminathan, Narasimman, Robinson, A. Karen, Ray, Amita, Autogenic drainage for airway clearance in cystic fibrosis, Cochrane Database of Systematic Reviews, 2012Cochrane systematic review - protocol only.
Thomas, J., Cook, D. J., Brooks, D., Chest physical therapy management of patients with cystic fibrosis. A meta-analysis, American Journal of Respiratory & Critical Care Medicine, 151, 846–50, 1995Low quality systematic review - 2/11 total score in AMSTAR checklist. Individual studies were checked for relevance.
Tonnesen, P., Kelstrup, M., Self-administered positive end-expiratory pressure (PEEP) employing a face mask as an alternative to conventional chest physiotherapy, Ugeskr-Laeg, 144, 1532–6, 1982Not English.
Van Asperen, P. P., Jackson, L., Hennessy, P., Brown, J., Comparison of a positive expiratory pressure (PEP) mask with postural drainage in patients with cystic fibrosis, Australian Paediatric Journal, 23, 283–4, 1987In Cochrane SR Main 2005. Study not included as the intervention (postural drainage) was not in the protocol.
van der Schans, C. P., van der Mark, T. W., de Vries, G., Piers, D. A., Beekhuis, H., Dankert-Roelse, J. E., Postma, D. S., Koeter, G. H., Effect of positive expiratory pressure breathing in patients with cystic fibrosis, Thorax, 46, 252–6, 1991In Cochrane SR Warnock 2013 (not analysed). Studies not analysed and below n = 10 excluded from review.
Van Ginderdeuren, F., Verbanck, S., Van Cauwelaert, K., Vanlaethem, S., Schuermans, D., Vincken, W., Malfroot, A., Chest physiotherapy in cystic fibrosis: short-term effects of autogenic drainage preceded by wet inhalation of saline versus autogenic drainage preceded by intrapulmonary percussive ventilation with saline, Respiration, 76, 175–80, 2008The comparison (same technique with the addition of saline) was not relevant to protocol.
Varekojis, S. M., Douce, F. H., Flucke, R. L., Filbrun, D. A., Tice, J. S., McCoy, K. S., Castile, R. G., A comparison of the therapeutic effectiveness of and preference for postural drainage and percussion, intrapulmonary percussive ventilation, and high-frequency chest wall compression in hospitalized cystic fibrosis patients, Respiratory Care, 48, 24–28, 2004The interventions were not included in the protocol (intrapulmonary ventilation, postural drainage percussion).
Webber, B, Parker, R, Hofmeyr, J, Hodson, M, Evaluation of self-percussion during postural drainage using the forced expiration technique, Physiother Pract, 1, 42–5., 1985The comparison was not prioritised for inclusion in the protocol (combination of self-percussion and PD vs PD alone).
Webber, Ba, Hofmeyr, Jl, Morgan, Md, Hodson, Me, Effects of postural drainage, incorporating the forced expiration technique, on pulmonary function in cystic fibrosis, Br J Dis Chest, 80, 353–9., 1986Not an RCT.
White, D, Stiller, K, Willson, K, The role of thoracic expansion exercises during the active cycle of breathing techniques, Physiotherapy Theory and Practice, 13, 155–62., 1997Intervention not relevant to the protocol (thoracic expansion exercises during ACBT).

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