Non‐invasive ventilation for cystic fibrosis

Fidelma Moran; Judy M Bradley; Amanda J Piper

doi:10.1002/14651858.CD002769.pub5

Non‐invasive ventilation for cystic fibrosis

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Referencias

References to studies included in this review

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Dwyer TJ, Robbins L, Kelly P, Piper AJ, Bell SC, Bye PT. 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 2015;61(3):142‐7. [CENTRAL: 1104106; CFGD Register: OV22; CRS: 5500135000001431; PUBMED: 26096013]

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Holland A, Denehey 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]. Proceedings of the American Thoracic Society 99th International Conference; 2003 May 16‐21; Seattle; USA. 2003:D041. [CFGD Register: PE143b]

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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:Abst #P140. [CFGD Register: PE143d]

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Kofler AM, Carlesi A, Cutrera R, Leone P, Lucidi V, Rosati S, et al. BiPAP versus PEP as chest physiotherapy in patients with cystic fibrosis [abstract]. Pediatric Pulmonology 1998;26(Suppl 17):344. [CFGD Register: PE95]

Lima C, De Andrade AD, Rattes C, Campos S, Brandao D, Aliverti A, et al. Effect of noninvasive ventilation on functional exercise capacity, lung function and compartmental chest wall volume in children with cystic fibrosis. European Respiratory Journal 2013;42 Suppl 57:P5065. [CENTRAL: 1099891; CFGD Register: PE216b ; CRS: 5500050000000267; EMBASE: 71843506]

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Milross MA, Piper AJ, Norman M, Becker HF, Willson GN, Grunstein RR, et al. Low‐flow oxygen and bilevel ventilatory support. American Journal of Respiratory and Critical Care Medicine 2001;163(1):129‐34. [CFGD Register: OV8b]

Milross MA, Piper AJ, Norman M, Willson GN, Becker HF, 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. [CFGD Register: OV8a]

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Placidi G, Cornacchia M, Polese G, Zanolla L, Assael B, 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. [CFGD Register: PE128b]

Rodriguez Hortal MC, Hjelte L. Non invasive ventilation as airway clearance technique compared to PEP in adult patients with cystic fibrosis [abstract]. Journal of Cystic Fibrosis 2013;12 Suppl 1:S18, Abstract no: WS9.4. [CENTRAL: 875000; CFGD Register: PE203a; CRS: 5500100000011654]

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Rodriguez Hortal MC, Nygren‐Bonnier M, Hjelte L. Non‐invasive ventilation as airway clearance technique in cystic fibrosis. Physiotherapy Research International 2016 Feb 29 [Epub ahead of print]. [CFGD Register: PE203b; DOI: 10.1002/pri.1667]

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. [CFGD Register: OV19c]

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. [CFGD Register: OV19b]

Young AC, Wilson JW, Kotsimbos TC, Naughton MT. Randomized placebo‐controlled trial of non‐invasive ventilation for hypercapnia in cystic fibrosis [abstract]. Proceedings of the Japanese Society of Sleep Research Conference. 2006:A9‐10. [CFGD Register: OV19a]

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References to studies excluded from this review

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Elkins MR, Eberl S, Alsion J, Bye P. The effect of bi‐level non‐invasive ventilation on mucociliary clearance in subjects with cystic fibrosis [abstract].. Pediatric Pulmonology. 2004; Vol. 38(Suppl 27):315. [CFGD Register: OV20]

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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. [CFGD Register: DT21]

Faurox B, Pigeot J, Isabey D, Harf A, Clèment A, Lofaso F. Two modes of non invasive mechanical ventilation can decrease the work of breathing in cystic fibrosis children with an acute exacerbation. European Respiratory Journal 2000;16(Suppl 31):15s. [CENTRAL: 415631; CFGD Register: OV23; CRS: 5500050000000270]

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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. [CFGD Register: PH157b]

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Greenough A, Limb E, Marlow N, Peacock JL, Calvert S. Radiological outcome of very prematurely born infants randomised to high frequency oscillatory or conventional ventilation. European Journal of Pediatrics 2004;163(11):671‐4.

Parreira V, Pires S, Sulmonett N, Camargos P, Haddad J, Britto R. Positive expiratory pressure and lung function in cystic fibrosis patients [abstract]. Proceedings of the European Respiratory Society Annual Congress; 2008 Oct 4‐8; Berlin, Germany.. 2008:E1779. [CENTRAL: 679902; CFGD Register: PE212; CRS: 5500050000000051]

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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. [CFGD Register: BD125]

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(Suppl 3):A555. [CFGD Register: OV16]

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References to studies awaiting assessment

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Petrone A, Quartieri M, Tirone F, Tirone C, Mauro GF. Management of patients with cystic fibrosis: Role of non invasive ventilation (NIV) and chest physiotherapy [abstract]. Proceedings of European Respiratory Society Annual Congress; 2010 Sep 18‐22; Barcelona, Spain. 2010:[E3907]. [CENTRAL: 777469; CFGD Register: PE220b; CRS: 5500050000000268]

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Additional references

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References to other published versions of this review

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Moran F, Bradley J. Non‐invasive ventilation for cystic fibrosis. Cochrane Database of Systematic Reviews 2003, Issue 2. [DOI: 10.1002/14651858.CD002769.pub2]

Moran F, Bradley JM, Jones AP, PiperAJ. Non‐invasive ventilation for cystic fibrosis. Cochrane Database of Systematic Reviews 2007, Issue 10. [DOI: 10.1002/14651858.CD002769.pub2]

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Characteristics of studies

Characteristics of included studies [ordered by study ID]

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Dwyer 2015

Methods	RCT. Parallel design. Duration: up to 2 weeks (during hospitalisation for an exacerbation). Location: 2 hospital sites.
Participants	40 adults with CF and an acute exacerbation (defined as needing intravenous antibiotics and four or more signs or symptoms from criteria by Fuchs (Fuchs 1994). Moderate to severe disease FEV₁ <60% predicted. NIV group Mean (SD) age: 28 (7) years. Gender n (%): 6 (32) females. BMI mean (SD): 21.4 (3.1) kg/m². FVC mean (SD): 61 (15) % predicted. PImax mean (SD): 87 (32) % predicted. PEmax mean (SD): 70 (22) % predicted. Control group Mean (SD) age: 30 (9) years. Gender n (%): 8 (38) females. BMI mean (SD): 20.4 (3.0) kg/m². FVC mean (SD): 63 (15) % predicted. PImax mean (SD): 93 (30) % predicted. PEmax mean (SD): 92 (41) % predicted.
Interventions	Intervention (n = 19): versus standard airway clearance + NIV delivered by a nasal mask or mouthpiece. Control: standard airway clearance. NIV: mean (SD) IPAP 13 cm H₂0 (2); mean (SD) EPAP: 5 cm H₂0 (1). Standard airway clearance = ACBT+ percussions; vibrations; postural drainage; some other techniques were also used for some treatments.
Outcomes	Primary outcome measure: change in FEV₁ % predicted from admission to discharge. Secondary outcome measures: visual analogue score; modified shuttle walk test; shortness of breath; sputum volume; energy levels; PImax, PEmax; quality of life (CFQ); quantitative sputum microbiology, length of admission; time to next hospital admission.
Notes
*Risk of bias*
Bias	Authors' judgement	Support for judgement
Random sequence generation (selection bias)	Low risk	"Computer‐generated block randomisation".
Allocation concealment (selection bias)	Low risk	Randomisation schedule was "... stored in sealed sequentially numbered opaque envelopes opened after consent signed".
Blinding (performance bias and detection bias) All outcomes	High risk	It is not possible to blind participants or assessors to the intervention.
Incomplete outcome data (attrition bias) All outcomes	Low risk	All participants accounted for. Three participants withdrew from the study and the data from these participants were used, as available and appropriate, in the analyses of daily rate of improvement, length of stay and time to next hospital admission.
Selective reporting (reporting bias)	Low risk	All the outcomes listed in the paper's methodology are the same as those presented in the results section of the full paper.
Other bias	Low risk	Methods of statistical analysis were described.

Fauroux 1999

Methods	RCT. Cross‐over design. Duration: single session.
Participants	16 participants with CF. Stable participants. Mean (SD) age 13 (4) years.
Interventions	Session 1: CPT (10 to 15 forced expiration manoeuvres separated by rest periods) and inspiratory PSV via nasal mask using pressure support generator. Session 2: CPT with no PSV. Sessions 20 minutes each but time between sessions unclear ‐ paper states sessions were conducted on 2 different days at the same time of day by same physiotherapist.
Outcomes	Lung function (FVC; FEV₁; PEF; FEF₂₅%; FEF₅₀%; FEF_25‐75%), airway resistance, SpO₂; RR; PImax; PEmax; sputum weight; subjective participants impressions of fatigue, ease sputum clearance; participant preference (1 = worse to 3 = marked preference).
Notes
*Risk of bias*
Bias	Authors' judgement	Support for judgement
Random sequence generation (selection bias)	Unclear risk	States order of intervention was randomised, but no details given.
Allocation concealment (selection bias)	Unclear risk	Not discussed.
Blinding (performance bias and detection bias) All outcomes	Unclear risk	Participants' subjective impressions were evaluated by individuals who were not involved in the trial and were unaware of the treatment regimen; but it was not reported who was responsible for collecting and weighing secretions and lung function testing.
Incomplete outcome data (attrition bias) All outcomes	Low risk	All participants were accounted for.
Selective reporting (reporting bias)	Low risk	All outcome measures were reported.
Other bias	Low risk	Methods of statistical analysis were described.

Gozal 1997

Methods	RCT. Cross‐over design. Duration: 3 nights within a 15‐day period.
Participants	Six participants with CF and moderate to severe lung disease and significant gas exchange abnormalities during sleep. Stable participants. Age mean (SD) [range]: 22.3 (4.7) [13 ‐ 28]. FEV₁% predicted mean (SD): 29.4% (3.4).
Interventions	Session 1: room air. Session 2: night‐time bilevel NIPPV with supplemental oxygen given via nasal mask.
Outcomes	TST; sleep latency; NREM; NREM %TST; REM min; REM %TST; undetermined % TST; total arousals; arousal index; SaO₂; TcCO₂.
Notes
*Risk of bias*
Bias	Authors' judgement	Support for judgement
Random sequence generation (selection bias)	Unclear risk	Stated as randomised, methods not discussed.
Allocation concealment (selection bias)	Unclear risk	Not discussed.
Blinding (performance bias and detection bias) All outcomes	Unclear risk	Participants were obviously aware of the intervention being administered; however, all were reportedly unaware of the purpose of the trial with participants and sleep technicians blinded to the results until completion of the third night.
Incomplete outcome data (attrition bias) All outcomes	Unclear risk	2 participants dropped out due to non‐tolerance of NIV; it was not explicitly stated that intention‐to‐treat was not used, but results are based on the remaining 6 participants who completed the trial.
Selective reporting (reporting bias)	Low risk	All outcome measures were reported.
Other bias	Unclear risk	Methods of statistical analysis were described.

Holland 2003

Methods	RCT (order of intervention was randomised on days 3 and 4 of hospital admission). Cross‐over design. Duration: single intervention of each.
Participants	26 participants with CF and moderate to severe disease. Acute participants. Mean (SD) age 27.04 (6.42) years. Mean (SD) FEV₁ % predicted 33.85 (11.85).
Interventions	Session 1: CPT by ACBT i.e. (thoracic expansion x6, breathing control) x2, FET and cough as required. Session 2: ACBT as above with NIV via nasal mask with heated humidification entrained. NIV range: IPAP 10 ‐ 12 cm H₂0; EPAP 4 ‐ 5 cm H₂0.
Outcomes	FVC; FEV₁; FEF_25‐75; PImax; PEmax; SpO₂; sputum weight; Borg breathlessness score; participant preference.
Notes
*Risk of bias*
Bias	Authors' judgement	Support for judgement
Random sequence generation (selection bias)	Unclear risk	States order of intervention was randomised, but no details given.
Allocation concealment (selection bias)	Unclear risk	Not discussed.
Blinding (performance bias and detection bias) All outcomes	Low risk	An independent data collector who was blinded to the treatment order obtained all measurements.
Incomplete outcome data (attrition bias) All outcomes	Low risk	Information provided about 1 drop out at the start of testing.
Selective reporting (reporting bias)	Low risk	All outcome measures were reported.
Other bias	Unclear risk	Methods of statistical analysis were described.

Kofler 1998

Methods	RCT. Cross‐over design. Duration: single sessions on 2 successive days, time between 2 sessions is 1 day.
Participants	20 participants with CF. No detail on whether participants are in acute or stable state, but participants have mean (SD) Swachman‐Kowalski score of 80.8 (15.3) indicating that they have mild disease. Mean (range) age 15.25 (6 ‐ 23) years.
Interventions	Session 1: PEP treatment (no details of PEP treatment). Session 2: bilevel positive airway pressure (BiPaP) treatment, type of interface unclear.
Outcomes	FEV₁; FVC; SaO₂; FEF_25‐75; MEF₅₀; FEF_25‐75; participant preference.
Notes
*Risk of bias*
Bias	Authors' judgement	Support for judgement
Random sequence generation (selection bias)	Unclear risk	States order of intervention was randomised, but no details given.
Allocation concealment (selection bias)	Unclear risk	Not discussed.
Blinding (performance bias and detection bias) All outcomes	Unclear risk	Data collection was not described.
Incomplete outcome data (attrition bias) All outcomes	Low risk	All participants were accounted for.
Selective reporting (reporting bias)	Unclear risk	Not clear whether all outcomes measured were reported in this abstract.
Other bias	Unclear risk	Methods of statistical analysis were not described.

Lima 2014

Methods	RCT. Cross‐over design. Duration: single session, each 24 to 48 hrs apart.
Participants	13 participants with CF. Mild, moderate and severe lung disease. Stable state with no history of hospitalisation for respiratory failure in previous 3 months. Mean age: 10.77 years. Mean FEV₁ % predicted: 62%. Mean BMI: 33.78 kg/m².
Interventions	Session 1: 6MWT with (type of interface unclear). Session 2: 6MWT with no NIV. 6MWT speed 2.5 km/h and increased every 30 seconds up to a maximum of 7 km/h. NIV: IPAP 12 cm H₂0; EPAP mean (SD) 6 (2) cm H₂0.
Outcomes	Walking distance, spirometry (FEV₁, FVC, FEF _25‐75), OEP (TV, MV, Vprc, Vab), RR, HR, SpO₂. OEP and spirometry measured before and 5 min after test. RR, HR and SpO₂ measured immediately before, immediately after and 5 min after test.
Notes
*Risk of bias*
Bias	Authors' judgement	Support for judgement
Random sequence generation (selection bias)	Low risk	Computer‐generated random permutated blocks.
Allocation concealment (selection bias)	Unclear risk	Not discussed.
Blinding (performance bias and detection bias) All outcomes	High risk	Open trial ‐ no evidence so no blinding of assessors, investigators or participants in the published report.
Incomplete outcome data (attrition bias) All outcomes	Low risk	All participants accounted for.
Selective reporting (reporting bias)	Low risk	Primary outcomes in protocol were reported. Some indices of secondary outcomes regarding rib cage volume P values reported in the abstract, but not reported within the full text i.e. VRCP, VRCA, and VAB.
Other bias	Low risk	Methods of statistical analysis were described.

Milross 2001

Methods	RCT. Cross‐over design. Duration: 3 nights within a 1‐week period, time between nights unclear.
Participants	13 participants with CF with severe lung disease. Mean (SD) age: 26 (5.9) years. Mean (SD) FEV₁ % predicted: 31.7% (10.6). Awake PaO₂ (range): 53 ‐ 77 mmHg. PaCO₂ ≥ 45 mmHg. BMI mean (SD): 20 (3) kg/m².
Interventions	Session 1: room air and low‐level CPAP (4 ‐ 5 cm H₂O). Session 2: oxygen (1.4 +/‐ 0.9L/min to maintain SaO₂ ≥ 90%) and low‐level CPAP (4 ‐ 5 cm H₂O) via a nasal mask. Session 3: BVS +/‐ oxygen (0.7+/‐0.9 L/min to maintain SaO₂ ≥ 90%).
Outcomes	VI, VT; RR; respiratory disturbance indices; SaO₂ TcCO₂.
Notes
*Risk of bias*
Bias	Authors' judgement	Support for judgement
Random sequence generation (selection bias)	Unclear risk	Stated as randomised, methods not discussed.
Allocation concealment (selection bias)	Unclear risk	Not discussed.
Blinding (performance bias and detection bias) All outcomes	High risk	No evidence of blinding of assessors, investigators or participants.
Incomplete outcome data (attrition bias) All outcomes	Low risk	No drop outs.
Selective reporting (reporting bias)	Low risk	All outcome measures were reported.
Other bias	Unclear risk	Methods of statistical analysis were described.

Placidi 2006

Methods	RCT. Cross‐over design. Duration: treatment 2x daily for 70 min for 2 days per intervention.
Participants	17 participants with CF. Severe lung disease. Acute participants. Mean (SD) age: 27 (7) years. FEV₁ % predicted mean (SD): 25% (6). BMI mean (SD): 18 (3) kg/m². MIP % predicted mean (SD): 87% (17). Wet weight sputum mean (SD): 5 (5) g.
Interventions	Intervention 1: directed cough; Intervention 2: PEP via nasal mask. Intervention 3: CPAP via nasal mask; Intervention for NIV with IPAP 8 ‐ 12 cm H₂O; EPAP 4 cm H₂O.
Outcomes	Sputum wet and dry weight; number spontaneous coughs; FEV₁; FVC; FEF; mean SpO₂; participants subjective impression of the effectiveness and fatigue induced by each treatment.
Notes
*Risk of bias*
Bias	Authors' judgement	Support for judgement
Random sequence generation (selection bias)	Low risk	Randomisation of treatment order was done according to the Latin square design which provided a balanced assignment to each treatment and a balance in the sequence of treatments.
Allocation concealment (selection bias)	Unclear risk	Not discussed.
Blinding (performance bias and detection bias) All outcomes	Unclear risk	Physiotherapists collected sputum and the technician was blinded to physiotherapy treatment but it is not identified who was responsible for weighing sputum or collating cough information induced by the treatment.
Incomplete outcome data (attrition bias) All outcomes	Unclear risk	All participants were accounted for.
Selective reporting (reporting bias)	Low risk	All outcome measures were reported.
Other bias	Unclear risk	Methods of statistical analysis were described.

Rodriguez Hortal 2016

Methods	RCT. Parallel design. Duration: both interventions given 2x daily for 60 min for 3 months.
Participants	32 participants with CF. Mean (SD) age: 31 (10) years. Moderate to severe lung disease. Baseline FEV₁ % predicted mean (SD) (range): 47% (14) (22% to 69%). Baseline FVC % predicted mean (SD): 69% (13).%.
Interventions	Intervention 1: treatment including bronchodilators; AD with HTS for 15 min; then NIV with face mask; FET/huffing from mid to low lung volume. Intervention 2: treatment including bronchodilators; AD with HTS for 15 min; then PEP with mask; FET/huffing from mid to low lung volume. NIV: IPAP 20 cm H₂O minimum; EPAP 10 cm H₂O. PEP: 10 breaths with 10 to 20 cm H₂O resistance.
Outcomes	Lung function (FEV₁; FVC; LCI), 6MWT, PaCO₂, sputum culture, inflammatory blood markers.
Notes
*Risk of bias*
Bias	Authors' judgement	Support for judgement
Random sequence generation (selection bias)	Unclear risk	States randomised but no further details given.
Allocation concealment (selection bias)	Low risk	States opaque envelopes were used to assign to 1 of 2 groups.
Blinding (performance bias and detection bias) All outcomes	Low risk	Pulmonary lab technicians blinded to physiotherapy treatment took lung function readings; research nurses blinded to treatment took vital signs; 6MWT was performed by the physiotherapist although it is unclear if the physiotherapist conducting the 6MWT is blinded to treatment allocation.
Incomplete outcome data (attrition bias) All outcomes	Low risk	All participants were accounted for.
Selective reporting (reporting bias)	Low risk	It is stated that inflammatory blood markers, blood gases and sputum analysis monthly was non‐significant compared to baseline (data not reported); FRC did not change in either group (data not reported). All other outcome measures were reported.
Other bias	Low risk	Methods of statistical analysis were reported.

Young 2008

Methods	RCT. Cross‐over design. Duration: each intervention for 6 weeks with 2‐week washout period.
Participants	8 participants with CF. Moderate and severe lung disease. No details on whether participants are in acute or stable state. Mean (SD) age: 37 (8) years. Mean (SD) FEV₁ % predicted: 35% (8). Mean (SD) BM:I 21.1 (2.6) kg/m². Mean (SD) PaCO₂: 52 (4) mmHg.
Interventions	Intervention 1: nocturnal air (placebo). Intervention 2: oxygen via nasal or full face mask. Intervention 2: NIV via nasal or full face mask.
Outcomes	CF‐specific QoL questionnaire; daytime sleepiness; exertional dyspnoea; awake and asleep gas exchange; sleep architecture; lung function; peak exercise capacity. Post‐treatment assessments were carried out during a period of clinical stability i.e. no current need for hospitalisation or intravenous antibiotics.
Notes	Also measured neurocognitive function (PVT :mean; error; lapse); Stroop Color & Word test; trail‐making test; controlled oral word association and digital span test which were reported in the online supplement. They are not reported in this review as they were not relevant to the aims of this review.
*Risk of bias*
Bias	Authors' judgement	Support for judgement
Random sequence generation (selection bias)	Low risk	Computer‐generated Latin square design.
Allocation concealment (selection bias)	Low risk	Sealed in sequentially numbered opaque envelopes by a person not involved in the trial and opened as each participant was enrolled.
Blinding (performance bias and detection bias) All outcomes	Unclear risk	Participants remained blinded as to whether they were receiving oxygen or room air only.
Incomplete outcome data (attrition bias) All outcomes	Low risk	One withdrawal after randomisation due to a pneumothorax. One withdrawal from NIV group due to mask discomfort (NIV n = 7; O₂ n = 8).
Selective reporting (reporting bias)	Low risk	All outcome measures were reported.
Other bias	Low risk	Methods of statistical analysis were described.

Full abbreviations list can be found in "Additional Tables" (Table 1)

Characteristics of excluded studies [ordered by study ID]

Ir a:

estudios incluidos
estudios a la espera de clasificación

Study	Reason for exclusion
Elkins 2004	This trial did not compare NIV to increase minute ventilation and is not linked to the outcome measures in this review.
Falk 2006	This trial did not use NIV.
Fauroux 2000a	This trial did not compare NIV with other management for acute or chronic respiratory failure.
Fauroux 2000b	This trial is not comparing NIV with other management strategies for acute or chronic respiratory failure.
Fauroux 2001	This is not a randomised controlled trial of NIV versus no NIV.
Fauroux 2004	This trial did not compare NIV with other management for acute or chronic respiratory failure.
Greenough 2004	This trial did not use NIV.
Parreira 2008	This trial did not use NIV pressure or volume preset mode.
Piper 1992	This is not a randomised controlled trial.
Regnis 1994	This is not a randomised controlled trial.
Riethmueller 2006	This trial did not use NIV.
Serra 2000	This is not a randomised controlled trial of NIV versus no NIV.
Serra 2002	This is not a randomised controlled trial of NIV versus no NIV.

see Table 1

Characteristics of studies awaiting assessment [ordered by study ID]

Ir a:

estudios incluidos
estudios excluidos

Petrone 2009

Methods	Participants divided into 3 random groups. Sleep studies performed on respiratory ward supervised by experienced nurses. Duration: 6 months treatment.
Participants	21 participants. Mean (SD) age: 27.3 (3.7) years. Mean (SD) FEV₁ % predicted: 44.1% (3.10).
Interventions	Group 1: ACBT. Group 2: ACBT with oxygen. Group 3: ACBT with NIV.
Outcomes	Frequency of respiratory exacerbations; arterial blood gases; spirometry; SWT; sleep parameters: AHI; mean SaO₂%; % of TST; oxygen desaturation index; SpO₂ mean % of TST; TcCO₂ mean of TST.
Notes	This is all the detail which is recorded in the abstract. To be completed once full study details are available.

see Table 1

Data and analyses

Open in table viewer

Comparison 1. NIV versus no NIV during chest physiotherapy (any technique)

Outcome or subgroup title	No. of studies	No. of participants	Statistical method	Effect size
1 CFQ scores Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 1.1 Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 1 CFQ scores.
1.1 Physical Domain	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
1.2 Health Domain	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
1.3 Respiratory Domain	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
2 Fatigue Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 1.2 Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 2 Fatigue.
3 FEV₁ (L) Show forest plot	2		Mean Difference (IV, Fixed, 95% CI)	Subtotals only
Open in figure viewer Analysis 1.3 Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 3 FEV₁ (L).
3.1 Up to 1 week (directed cough)	2	86	Mean Difference (IV, Fixed, 95% CI)	‐0.03 [‐0.17, 0.12]
3.2 Up to 1 week (PEP)	1	34	Mean Difference (IV, Fixed, 95% CI)	‐0.05 [‐0.22, 0.12]
4 FEV₁ (% predicted) Show forest plot	3		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 1.4 Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 4 FEV₁ (% predicted).
4.1 Up to 1 week (directed cough)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
4.2 Up to 2 weeks (all techniques combined)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
4.3 Up to 3 months (PEP)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
5 FVC (L) Show forest plot	2		Mean Difference (IV, Fixed, 95% CI)	Subtotals only
Open in figure viewer Analysis 1.5 Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 5 FVC (L).
5.1 Up to 1 week (directed cough)	2	86	Mean Difference (IV, Fixed, 95% CI)	0.00 [‐0.30, 0.30]
5.2 Up to 1 week (PEP)	1	34	Mean Difference (IV, Fixed, 95% CI)	‐0.07 [‐0.47, 0.33]
6 FVC (% predicted) Show forest plot	2		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 1.6 Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 6 FVC (% predicted).
6.1 Up to 1 week (directed cough)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
6.2 Up to 3 months (PEP)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
7 FEF_25‐75 (L) Show forest plot	2		Mean Difference (IV, Fixed, 95% CI)	Subtotals only
Open in figure viewer Analysis 1.7 Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 7 FEF_25‐75 (L).
7.1 Up to 1 week (directed cough)	2	86	Mean Difference (IV, Fixed, 95% CI)	‐0.00 [‐0.08, 0.07]
7.2 Up to 1 week (PEP)	1	34	Mean Difference (IV, Fixed, 95% CI)	0.0 [‐0.07, 0.07]
8 FEF_25‐75 (% predicted) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 1.8 Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 8 FEF_25‐75 (% predicted).
8.1 Up to 1 week (directed cough)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
9 Respiratory muscle strength PImax (cmH₂0) Show forest plot	2		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 1.9 Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 9 Respiratory muscle strength PImax (cmH₂0).
9.1 Up to 1 week (all techniques combined)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
9.2 Up to 2 weeks (all techniques combined)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
10 Respiratory muscle strength PEmax (cmH₂0) Show forest plot	2		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 1.10 Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 10 Respiratory muscle strength PEmax (cmH₂0).
10.1 Up to 1 week (all techniques combined)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
10.2 Up to 2 weeks (all techniques combined)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
11 Airway resistance % predicted Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 1.11 Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 11 Airway resistance % predicted.
11.1 Up to 1 week (all techniques combined)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
12 LCI Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 1.12 Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 12 LCI.
12.1 Up to 3 months (PEP)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
13 Oxygen saturation during airway clearance (%) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 1.13 Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 13 Oxygen saturation during airway clearance (%).
13.1 Up to 1 week (all techniques combined)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
14 Oxygen saturation during airway clearance (change in SpO₂ % during treatment) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 1.14 Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 14 Oxygen saturation during airway clearance (change in SpO₂ % during treatment).
14.1 Up to 1 week (all techniques combined)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
15 Oxygen saturation after airway clearance (SpO₂) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 1.15 Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 15 Oxygen saturation after airway clearance (SpO₂).
15.1 Up to 1 week (directed cough)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
15.2 Up to 1 week (PEP)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
16 PaCO2 Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 1.16 Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 16 PaCO2.
16.1 Up to 3 months (PEP)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
17 Breathlessness Show forest plot	2		Std. Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 1.17 Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 17 Breathlessness.
17.1 Up to 1 week (Borg breathlessness score)	1		Std. Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
17.2 Up to 2 weeks (VAS score)	1		Std. Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
18 Sputum wet weight (g) Show forest plot	3		Mean Difference (IV, Fixed, 95% CI)	Subtotals only
Open in figure viewer Analysis 1.18 Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 18 Sputum wet weight (g).
18.1 Up to 1 week (directed cough)	3	118	Mean Difference (IV, Fixed, 95% CI)	‐0.69 [‐3.06, 1.67]
18.2 Up to 1 week (PEP)	1	34	Mean Difference (IV, Fixed, 95% CI)	‐2.58 [‐6.11, 0.95]
19 Sputum dry weight (g) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 1.19 Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 19 Sputum dry weight (g).
19.1 Up to 1 week (directed cough)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
19.2 Up to 1 week (PEP)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
20 Sputum volume per day (VAS score) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 1.20 Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 20 Sputum volume per day (VAS score).
20.1 Up to 2 weeks (all techniques combined)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
21 Pseudomonas density (log CFU/g) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 1.21 Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 21 Pseudomonas density (log CFU/g).
21.1 Up to 2 weeks (all techniques combined)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
22 Exercise capacity: 25 level modified shuttle test (m) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 1.22 Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 22 Exercise capacity: 25 level modified shuttle test (m).
22.1 Up to 2 weeks (all techniques combined)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
23 Energy (VAS score) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 1.23 Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 23 Energy (VAS score).
23.1 Up to 2 weeks (all techniques combined)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
24 6MWT Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 1.24 Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 24 6MWT.
24.1 Up to 3 months (PEP)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
25 Length of hospital stay (days) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 1.25 Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 25 Length of hospital stay (days).
25.1 Up to 2 weeks (all techniques combined)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
26 Time to next admission (days) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 1.26 Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 26 Time to next admission (days).

Open in table viewer

Comparison 2. NIV in overnight ventilation compared to oxygen

Outcome or subgroup title	No. of studies	No. of participants	Statistical method	Effect size
1 CFQoL chest symptom score Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 2.1 Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 1 CFQoL chest symptom score.
1.1 Up to 3 months	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
2 CFQoL transitional dyspnoea index Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 2.2 Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 2 CFQoL transitional dyspnoea index.
2.1 Up to 3 months	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
3 Symptoms of sleep‐disordered breathing Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 2.3 Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 3 Symptoms of sleep‐disordered breathing.
3.1 Epworth Sleepiness Scale (up to 3 months)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
3.2 Global PSQI (up to 3 months)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
4 Lung function during sleep Show forest plot	1		Mean Difference (Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 2.4 Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 4 Lung function during sleep.
4.1 VI (L/m) while awake (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
4.2 VI (L/m) during REM (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
4.3 VI (L/m) during NREM (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
4.4 VT (L) while awake (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
4.5 VT (L) during REM (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
4.6 VT (L) during NREM (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
5 Respiratory rate (breaths/min) Show forest plot	1		Mean Difference (Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 2.5 Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 5 Respiratory rate (breaths/min).
5.1 RR while awake (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
5.2 RR during REM (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
5.3 RR during NREM (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
6 Lung function while awake Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 2.6 Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 6 Lung function while awake.
6.1 FEV1% predicted (up to 3 months)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
6.2 FVC % predicted (up to 3 months)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
7 Arterial blood gases: pH Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 2.7 Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 7 Arterial blood gases: pH.
7.1 Up to 3 months	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
8 Arterial blood gases: PaO₂ (mmHg) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 2.8 Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 8 Arterial blood gases: PaO₂ (mmHg).
8.1 Up to 3 months	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
9 Arterial blood gases: PaCO₂ (mmHg) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 2.9 Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 9 Arterial blood gases: PaCO₂ (mmHg).
9.1 Up to 3 months	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
10 Arterial blood gases: HCO₃ (mmol/L) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 2.10 Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 10 Arterial blood gases: HCO₃ (mmol/L).
10.1 Up to 3 months	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
11 Arterial blood gases: SaO₂ (%) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 2.11 Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 11 Arterial blood gases: SaO₂ (%).
11.1 Up to 3 months	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
12 Exercise performance (MSWT) (metres) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 2.12 Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 12 Exercise performance (MSWT) (metres).
12.1 Up to 3 months	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
13 Total sleep time (min) Show forest plot	2		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 2.13 Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 13 Total sleep time (min).
13.1 Single night	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
13.2 Up to 3 months	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
14 REM sleep architecture Show forest plot	2		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 2.14 Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 14 REM sleep architecture.
14.1 REM (single night)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
14.2 REM %TST (single night)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
14.3 REM % TST (up to 3 months)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
15 Sleep latency (min) Show forest plot	3		Mean Difference (IV, Fixed, 95% CI)	Subtotals only
Open in figure viewer Analysis 2.15 Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 15 Sleep latency (min).
15.1 Single night	2	38	Mean Difference (IV, Fixed, 95% CI)	2.93 [‐0.32, 6.19]
15.2 Up to 3 months	1	15	Mean Difference (IV, Fixed, 95% CI)	‐5.0 [‐19.17, 9.17]
16 Nocturnal oxygen saturation (%) Show forest plot	2		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 2.16 Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 16 Nocturnal oxygen saturation (%).
16.1 Mean SpO2 REM (single night)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
16.2 Mean SpO2 NREM (single night)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
16.3 Mean SpO2 for TST (up to 3 months)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
16.4 TST with SpO2 < 90% (up to 3 months)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
17 Nocturnal oxygen saturation (%) Show forest plot	1		Mean Difference (Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 2.17 Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 17 Nocturnal oxygen saturation (%).
17.1 Percentage TST SpO2>90%	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
17.2 Percentage REM SpO2>90%	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
17.3 Percentage NREM SpO2>90%	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
18 Nocturnal TcCO₂ (mmHg Show forest plot	2		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 2.18 Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 18 Nocturnal TcCO₂ (mmHg.
18.1 TcCO2 during REM (single night)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
18.2 TcCO2 during NREM (single night)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
18.3 Mean change PtCO2 (mmHg) (up to 3 months)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
18.4 Mean change PaCO2 (mmHg) (up to 3 months)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
19 Nocturnal TcCO₂ (mmHg) Show forest plot	1		Mean Difference (Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 2.19 Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 19 Nocturnal TcCO₂ (mmHg).
19.1 Mean change TcCO2 NREM to REM (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
19.2 Maximum TcCO2 (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
20 Nocturnal TcCO₂ TST (mmHg) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 2.20 Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 20 Nocturnal TcCO₂ TST (mmHg).
20.1 Mean PtCO2 TST (up to 3 months)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
20.2 Maximum PtCO2 TST (up to 3 months)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
21 Hypopneas Show forest plot	1		Relative rate (Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 2.21 Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 21 Hypopneas.
21.1 Single night	1		Relative rate (Fixed, 95% CI)	0.0 [0.0, 0.0]
22 Mean heart rate (beats/min) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 2.22 Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 22 Mean heart rate (beats/min).
22.1 Up to 3 months	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
23 Respiratory rate Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 2.23 Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 23 Respiratory rate.
23.1 Up to 3 months	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]

Open in table viewer

Comparison 3. NIV in overnight ventilation compared to room air

Outcome or subgroup title	No. of studies	No. of participants	Statistical method	Effect size
1 CF QoL chest symptom score Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 3.1 Comparison 3 NIV in overnight ventilation compared to room air, Outcome 1 CF QoL chest symptom score.
1.1 Up to 3 months	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
2 CF QoL traditional dyspnoea index score Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 3.2 Comparison 3 NIV in overnight ventilation compared to room air, Outcome 2 CF QoL traditional dyspnoea index score.
2.1 Up to 3 months	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
3 Symptoms of sleep‐disordered breathing Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 3.3 Comparison 3 NIV in overnight ventilation compared to room air, Outcome 3 Symptoms of sleep‐disordered breathing.
3.1 Epworth sleepiness scale (up to 3 months)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
3.2 Global PSQI (up to 3 months)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
4 Lung function during sleep Show forest plot	1		Mean Difference (Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 3.4 Comparison 3 NIV in overnight ventilation compared to room air, Outcome 4 Lung function during sleep.
4.1 VI while awake (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
4.2 VI during REM (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
4.3 VI during NREM (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
4.4 VT while awake (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
4.5 VT during REM (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
4.6 VT during NREM (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
5 Respiratory rate(breaths/min) during sleep Show forest plot	1		Mean Difference (Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 3.5 Comparison 3 NIV in overnight ventilation compared to room air, Outcome 5 Respiratory rate(breaths/min) during sleep.
5.1 RR while awake (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
5.2 RR during REM (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
5.3 RR during NREM (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
6 Mean Respiratory Rate Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 3.6 Comparison 3 NIV in overnight ventilation compared to room air, Outcome 6 Mean Respiratory Rate.
6.1 Up to 3 months	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
7 Mean Heart Rate (beats/min) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 3.7 Comparison 3 NIV in overnight ventilation compared to room air, Outcome 7 Mean Heart Rate (beats/min).
7.1 Up to 3 months	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
8 Lung function while awake Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 3.8 Comparison 3 NIV in overnight ventilation compared to room air, Outcome 8 Lung function while awake.
8.1 FEV1% predicted (up to 3 months)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
8.2 FVC % predicted (up to 3 months)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
9 Arterial blood gases: pH Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 3.9 Comparison 3 NIV in overnight ventilation compared to room air, Outcome 9 Arterial blood gases: pH.
9.1 Up to 3 months	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
10 Arterial blood gases: PaO₂ (mmHg) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 3.10 Comparison 3 NIV in overnight ventilation compared to room air, Outcome 10 Arterial blood gases: PaO₂ (mmHg).
10.1 Up to 3 months	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
11 Arterial blood gases: PaCO₂ (mmHg) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 3.11 Comparison 3 NIV in overnight ventilation compared to room air, Outcome 11 Arterial blood gases: PaCO₂ (mmHg).
11.1 Up to 3 months	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
12 Arterial blood gases: HCO₃ (mmol/L) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 3.12 Comparison 3 NIV in overnight ventilation compared to room air, Outcome 12 Arterial blood gases: HCO₃ (mmol/L).
12.1 Up to 3 months	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
13 Arterial blood gases: SaO2 (%) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 3.13 Comparison 3 NIV in overnight ventilation compared to room air, Outcome 13 Arterial blood gases: SaO2 (%).
13.1 Up to 3 months	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
14 Exercise performance (metres) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 3.14 Comparison 3 NIV in overnight ventilation compared to room air, Outcome 14 Exercise performance (metres).
14.1 Up to 3 months	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
15 Total sleep time (min) Show forest plot	2		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 3.15 Comparison 3 NIV in overnight ventilation compared to room air, Outcome 15 Total sleep time (min).
15.1 Single night	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
15.2 Up to 3 months	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
16 REM sleep architecture Show forest plot	2		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 3.16 Comparison 3 NIV in overnight ventilation compared to room air, Outcome 16 REM sleep architecture.
16.1 REM (single night)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
16.2 REM %TST (single night)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
16.3 REM % TST (up to 3 months)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
17 Sleep latency Show forest plot	3		Mean Difference (IV, Fixed, 95% CI)	Subtotals only
Open in figure viewer Analysis 3.17 Comparison 3 NIV in overnight ventilation compared to room air, Outcome 17 Sleep latency.
17.1 Single night	2	38	Mean Difference (IV, Fixed, 95% CI)	‐2.63 [‐7.37, 2.11]
17.2 At 6 weeks	1	15	Mean Difference (IV, Fixed, 95% CI)	‐3.00 [‐19.88, 13.88]
18 Nocturnal oxygen saturation (%) Show forest plot	2		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 3.18 Comparison 3 NIV in overnight ventilation compared to room air, Outcome 18 Nocturnal oxygen saturation (%).
18.1 Mean SpO2 REM (single night)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
18.2 Mean SpO2 NREM (single night)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
18.3 Mean SpO2 for TST (up to 3 months)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
18.4 TST for SpO2 < 90% (up to 3 months)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
19 Nocturnal oxygen saturation (%) Show forest plot	1		Mean Difference (Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 3.19 Comparison 3 NIV in overnight ventilation compared to room air, Outcome 19 Nocturnal oxygen saturation (%).
19.1 Percentage TST SpO2 > 90% (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
19.2 Percentage REM SpO2 > 90% (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
19.3 Percentage NREM SpO2 > 90% (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
20 Nocturnal TcCO₂ (mmHg) Show forest plot	2		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 3.20 Comparison 3 NIV in overnight ventilation compared to room air, Outcome 20 Nocturnal TcCO₂ (mmHg).
20.1 TcCO2 during REM (single night)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
20.2 TcCO2 during NREM (single night)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
20.3 Mean change PtCO2 (up to 3 months)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
20.4 Mean change PaCO2 (up to 3 months)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
21 Nocturnal TcCO₂(mmHg) Show forest plot	1		Mean Difference (Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 3.21 Comparison 3 NIV in overnight ventilation compared to room air, Outcome 21 Nocturnal TcCO₂(mmHg).
21.1 Mean change TcCO2 NREM to REM (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
21.2 Maximum TcCO2 (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
22 Nocturnal TcCO₂ TST (mmHg) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 3.22 Comparison 3 NIV in overnight ventilation compared to room air, Outcome 22 Nocturnal TcCO₂ TST (mmHg).
22.1 Mean PtCO2 TST (up to 3 months)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
22.2 Max PtCO2 TST (up to 3 months)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
23 Hypopneas Show forest plot	1		Relative rate (Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 3.23 Comparison 3 NIV in overnight ventilation compared to room air, Outcome 23 Hypopneas.
23.1 Single night	1		Relative rate (Fixed, 95% CI)	0.0 [0.0, 0.0]

Open in table viewer

Comparison 4. NIV versus no NIV during exercise testing

Outcome or subgroup title	No. of studies	No. of participants	Statistical method	Effect size
1 6 minute walk test Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected
Open in figure viewer Analysis 4.1 Comparison 4 NIV versus no NIV during exercise testing, Outcome 1 6 minute walk test.
1.1 Up to 1 week	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]

Analysis 1.1

Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 1 CFQ scores.

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Analysis 1.2

Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 2 Fatigue.

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Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 3 FEV1 (L).

Analysis 1.3

Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 3 FEV₁ (L).

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Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 4 FEV1 (% predicted).

Analysis 1.4

Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 4 FEV₁ (% predicted).

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Analysis 1.5

Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 5 FVC (L).

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Analysis 1.6

Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 6 FVC (% predicted).

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Analysis 1.7

Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 7 FEF_25‐75 (L).

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Analysis 1.8

Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 8 FEF_25‐75 (% predicted).

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Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 9 Respiratory muscle strength PImax (cmH20).

Analysis 1.9

Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 9 Respiratory muscle strength PImax (cmH₂0).

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Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 10 Respiratory muscle strength PEmax (cmH20).

Analysis 1.10

Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 10 Respiratory muscle strength PEmax (cmH₂0).

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Analysis 1.11

Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 11 Airway resistance % predicted.

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Analysis 1.12

Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 12 LCI.

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Analysis 1.13

Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 13 Oxygen saturation during airway clearance (%).

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Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 14 Oxygen saturation during airway clearance (change in SpO2 % during treatment).

Analysis 1.14

Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 14 Oxygen saturation during airway clearance (change in SpO₂ % during treatment).

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Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 15 Oxygen saturation after airway clearance (SpO2).

Analysis 1.15

Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 15 Oxygen saturation after airway clearance (SpO₂).

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Analysis 1.16

Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 16 PaCO2.

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Analysis 1.17

Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 17 Breathlessness.

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Analysis 1.18

Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 18 Sputum wet weight (g).

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Analysis 1.19

Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 19 Sputum dry weight (g).

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Analysis 1.20

Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 20 Sputum volume per day (VAS score).

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Analysis 1.21

Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 21 Pseudomonas density (log CFU/g).

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Analysis 1.22

Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 22 Exercise capacity: 25 level modified shuttle test (m).

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Analysis 1.23

Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 23 Energy (VAS score).

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Analysis 1.24

Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 24 6MWT.

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Analysis 1.25

Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 25 Length of hospital stay (days).

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Analysis 1.26

Comparison 1 NIV versus no NIV during chest physiotherapy (any technique), Outcome 26 Time to next admission (days).

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Analysis 2.1

Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 1 CFQoL chest symptom score.

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Analysis 2.2

Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 2 CFQoL transitional dyspnoea index.

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Analysis 2.3

Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 3 Symptoms of sleep‐disordered breathing.

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Analysis 2.4

Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 4 Lung function during sleep.

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Analysis 2.5

Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 5 Respiratory rate (breaths/min).

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Analysis 2.6

Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 6 Lung function while awake.

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Analysis 2.7

Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 7 Arterial blood gases: pH.

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Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 8 Arterial blood gases: PaO2 (mmHg).

Analysis 2.8

Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 8 Arterial blood gases: PaO₂ (mmHg).

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Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 9 Arterial blood gases: PaCO2 (mmHg).

Analysis 2.9

Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 9 Arterial blood gases: PaCO₂ (mmHg).

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Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 10 Arterial blood gases: HCO3 (mmol/L).

Analysis 2.10

Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 10 Arterial blood gases: HCO₃ (mmol/L).

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Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 11 Arterial blood gases: SaO2 (%).

Analysis 2.11

Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 11 Arterial blood gases: SaO₂ (%).

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Analysis 2.12

Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 12 Exercise performance (MSWT) (metres).

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Analysis 2.13

Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 13 Total sleep time (min).

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Analysis 2.14

Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 14 REM sleep architecture.

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Analysis 2.15

Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 15 Sleep latency (min).

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Analysis 2.16

Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 16 Nocturnal oxygen saturation (%).

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Analysis 2.17

Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 17 Nocturnal oxygen saturation (%).

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Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 18 Nocturnal TcCO2 (mmHg.

Analysis 2.18

Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 18 Nocturnal TcCO₂ (mmHg.

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Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 19 Nocturnal TcCO2 (mmHg).

Analysis 2.19

Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 19 Nocturnal TcCO₂ (mmHg).

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Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 20 Nocturnal TcCO2 TST (mmHg).

Analysis 2.20

Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 20 Nocturnal TcCO₂ TST (mmHg).

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Analysis 2.21

Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 21 Hypopneas.

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Analysis 2.22

Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 22 Mean heart rate (beats/min).

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Analysis 2.23

Comparison 2 NIV in overnight ventilation compared to oxygen, Outcome 23 Respiratory rate.

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Analysis 3.1

Comparison 3 NIV in overnight ventilation compared to room air, Outcome 1 CF QoL chest symptom score.

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Analysis 3.2

Comparison 3 NIV in overnight ventilation compared to room air, Outcome 2 CF QoL traditional dyspnoea index score.

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Analysis 3.3

Comparison 3 NIV in overnight ventilation compared to room air, Outcome 3 Symptoms of sleep‐disordered breathing.

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Analysis 3.4

Comparison 3 NIV in overnight ventilation compared to room air, Outcome 4 Lung function during sleep.

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Analysis 3.5

Comparison 3 NIV in overnight ventilation compared to room air, Outcome 5 Respiratory rate(breaths/min) during sleep.

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Analysis 3.6

Comparison 3 NIV in overnight ventilation compared to room air, Outcome 6 Mean Respiratory Rate.

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Analysis 3.7

Comparison 3 NIV in overnight ventilation compared to room air, Outcome 7 Mean Heart Rate (beats/min).

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Analysis 3.8

Comparison 3 NIV in overnight ventilation compared to room air, Outcome 8 Lung function while awake.

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Analysis 3.9

Comparison 3 NIV in overnight ventilation compared to room air, Outcome 9 Arterial blood gases: pH.

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Comparison 3 NIV in overnight ventilation compared to room air, Outcome 10 Arterial blood gases: PaO2 (mmHg).

Analysis 3.10

Comparison 3 NIV in overnight ventilation compared to room air, Outcome 10 Arterial blood gases: PaO₂ (mmHg).

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Comparison 3 NIV in overnight ventilation compared to room air, Outcome 11 Arterial blood gases: PaCO2 (mmHg).

Analysis 3.11

Comparison 3 NIV in overnight ventilation compared to room air, Outcome 11 Arterial blood gases: PaCO₂ (mmHg).

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Comparison 3 NIV in overnight ventilation compared to room air, Outcome 12 Arterial blood gases: HCO3 (mmol/L).

Analysis 3.12

Comparison 3 NIV in overnight ventilation compared to room air, Outcome 12 Arterial blood gases: HCO₃ (mmol/L).

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Analysis 3.13

Comparison 3 NIV in overnight ventilation compared to room air, Outcome 13 Arterial blood gases: SaO2 (%).

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Analysis 3.14

Comparison 3 NIV in overnight ventilation compared to room air, Outcome 14 Exercise performance (metres).

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Analysis 3.15

Comparison 3 NIV in overnight ventilation compared to room air, Outcome 15 Total sleep time (min).

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Analysis 3.16

Comparison 3 NIV in overnight ventilation compared to room air, Outcome 16 REM sleep architecture.

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Analysis 3.17

Comparison 3 NIV in overnight ventilation compared to room air, Outcome 17 Sleep latency.

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Analysis 3.18

Comparison 3 NIV in overnight ventilation compared to room air, Outcome 18 Nocturnal oxygen saturation (%).

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Analysis 3.19

Comparison 3 NIV in overnight ventilation compared to room air, Outcome 19 Nocturnal oxygen saturation (%).

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Comparison 3 NIV in overnight ventilation compared to room air, Outcome 20 Nocturnal TcCO2 (mmHg).

Analysis 3.20

Comparison 3 NIV in overnight ventilation compared to room air, Outcome 20 Nocturnal TcCO₂ (mmHg).

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Comparison 3 NIV in overnight ventilation compared to room air, Outcome 21 Nocturnal TcCO2(mmHg).

Analysis 3.21

Comparison 3 NIV in overnight ventilation compared to room air, Outcome 21 Nocturnal TcCO₂(mmHg).

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Comparison 3 NIV in overnight ventilation compared to room air, Outcome 22 Nocturnal TcCO2 TST (mmHg).

Analysis 3.22

Comparison 3 NIV in overnight ventilation compared to room air, Outcome 22 Nocturnal TcCO₂ TST (mmHg).

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Analysis 3.23

Comparison 3 NIV in overnight ventilation compared to room air, Outcome 23 Hypopneas.

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Analysis 4.1

Comparison 4 NIV versus no NIV during exercise testing, Outcome 1 6 minute walk test.

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Table 1. List of abbreviations

Abbreviation	Definition	Explanation
6MWT	six minute walk test
ABG	analysis of arterial blood gases
ACBT	active cycle of breathing technique
AHI	apnoea/hypopnoea Index
CF	cystic fibrosis
COPD	chronic obstructive pulmonary disease
CPAP	continuous positive airway pressure	a system that maintains a positive pressure in the circuitry and airways throughout inspiration and expiration
CPT	chest physiotherapy
CSS	chest symptom score	a validated CF quality of life measurement on a scale of 0 (worst) to 100 (best)
ESS	Epworth sleepiness scale	a scale measuring sleepiness ranging from 0 (best) to 24 (worst)
FEF_25‐75	flow rate between 25% and 75% of maximal expiration
FEV₁	forced expiratory volume in 1 second
FRC	functional residual capacity	resting volume of the lungs
FVC	forced vital capacity	total volume of air expired during a forced expiration following a full inspiration
Global PSQI	Global score Pittsburgh sleep quality index	sleep quality scale ranging from 0 (best) to 21 (worst)
LCI	lung clearance index	measured by multiple breath washouts; a sensitive measure of ventilation inhomogeneity
MEF₅₀	maximal expiratory flow with 50% of vital capacity remaining in the lung
mmHg	millimetres of mercury
mSpO₂	mean oxygen saturation
MSWT	modified shuttle walk test	incremental exercise tolerance test with minimum clinically important difference = 40 m
nadirSpO₂	the largest fall expressed in the absolute value of SpO₂
NIPPV	non‐invasive positive pressure ventilation
NIV	non‐invasive ventilation
NREM	non‐rapid eye movement	a phase during sleep
ODI	oxygen desaturation index
OEP	optoelectronic plethysmography
PaCO₂	partial pressure of carbon dioxide in arterial blood
PaO₂	partial pressure of oxygen in arterial blood
PEP	positive expiratory pressure	an airway clearance technique
PSV	pressure support ventilation
QoL	quality of life
RCT	randomised controlled trial
RDI	respiratory disturbance index
RE	respiratory exacerbations
REM	rapid eye movement	a phase during sleep
RR	respiratory rate
SaO₂	saturation of haemoglobin with oxygen in arterial blood
SD	standard deviation
SOB	shortness of breath
SpO₂	saturation of haemoglobin with oxygen using pulse oximetry
*SpO₂ max	the largest fall expressed as the difference with the SpO₂ just before the manoeuvre
*SpO₂ mean	the mean of *SpO₂ max during the whole chest physiotherapy period
SWT	shuttle walk test
TcCO₂	transcutaneous carbon dioxide
TDI	transitional dyspnoea index	a measurement of CF quality of life, scores ranging from ‐9 (worst) to +9 (best); the minimal clinically important difference is 1 unit
TLC	total lung capacity	total volume of air in lungs following a maximum inspiration
TST	total sleep time
TWT	treadmill walking test
VI	minute ventilation
VT	tidal volume	volume air inspired or expired during normal breathing
IPAP	inspiratory positive airway pressure
PImax	inspiratory respiratory muscle strength
PEmax	expiratory respiratory muscle strength
Vab	abdominal volume of the chest wall	variable calculated from OEP
Vrca	abdominal rib cage volume	variable calculated from OEP
Vrcp	pulmonary rib cage volume	variable calculated from OEP

Table 1. List of abbreviations

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Comparison 1. NIV versus no NIV during chest physiotherapy (any technique)

Outcome or subgroup title	No. of studies	No. of participants	Statistical method	Effect size
1 CFQ scores Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

1.1 Physical Domain	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
1.2 Health Domain	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
1.3 Respiratory Domain	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
2 Fatigue Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

3 FEV₁ (L) Show forest plot	2		Mean Difference (IV, Fixed, 95% CI)	Subtotals only

3.1 Up to 1 week (directed cough)	2	86	Mean Difference (IV, Fixed, 95% CI)	‐0.03 [‐0.17, 0.12]
3.2 Up to 1 week (PEP)	1	34	Mean Difference (IV, Fixed, 95% CI)	‐0.05 [‐0.22, 0.12]
4 FEV₁ (% predicted) Show forest plot	3		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

4.1 Up to 1 week (directed cough)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
4.2 Up to 2 weeks (all techniques combined)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
4.3 Up to 3 months (PEP)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
5 FVC (L) Show forest plot	2		Mean Difference (IV, Fixed, 95% CI)	Subtotals only

5.1 Up to 1 week (directed cough)	2	86	Mean Difference (IV, Fixed, 95% CI)	0.00 [‐0.30, 0.30]
5.2 Up to 1 week (PEP)	1	34	Mean Difference (IV, Fixed, 95% CI)	‐0.07 [‐0.47, 0.33]
6 FVC (% predicted) Show forest plot	2		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

6.1 Up to 1 week (directed cough)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
6.2 Up to 3 months (PEP)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
7 FEF_25‐75 (L) Show forest plot	2		Mean Difference (IV, Fixed, 95% CI)	Subtotals only

7.1 Up to 1 week (directed cough)	2	86	Mean Difference (IV, Fixed, 95% CI)	‐0.00 [‐0.08, 0.07]
7.2 Up to 1 week (PEP)	1	34	Mean Difference (IV, Fixed, 95% CI)	0.0 [‐0.07, 0.07]
8 FEF_25‐75 (% predicted) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

8.1 Up to 1 week (directed cough)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
9 Respiratory muscle strength PImax (cmH₂0) Show forest plot	2		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

9.1 Up to 1 week (all techniques combined)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
9.2 Up to 2 weeks (all techniques combined)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
10 Respiratory muscle strength PEmax (cmH₂0) Show forest plot	2		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

10.1 Up to 1 week (all techniques combined)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
10.2 Up to 2 weeks (all techniques combined)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
11 Airway resistance % predicted Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

11.1 Up to 1 week (all techniques combined)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
12 LCI Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

12.1 Up to 3 months (PEP)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
13 Oxygen saturation during airway clearance (%) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

13.1 Up to 1 week (all techniques combined)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
14 Oxygen saturation during airway clearance (change in SpO₂ % during treatment) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

14.1 Up to 1 week (all techniques combined)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
15 Oxygen saturation after airway clearance (SpO₂) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

15.1 Up to 1 week (directed cough)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
15.2 Up to 1 week (PEP)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
16 PaCO2 Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

16.1 Up to 3 months (PEP)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
17 Breathlessness Show forest plot	2		Std. Mean Difference (IV, Fixed, 95% CI)	Totals not selected

17.1 Up to 1 week (Borg breathlessness score)	1		Std. Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
17.2 Up to 2 weeks (VAS score)	1		Std. Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
18 Sputum wet weight (g) Show forest plot	3		Mean Difference (IV, Fixed, 95% CI)	Subtotals only

18.1 Up to 1 week (directed cough)	3	118	Mean Difference (IV, Fixed, 95% CI)	‐0.69 [‐3.06, 1.67]
18.2 Up to 1 week (PEP)	1	34	Mean Difference (IV, Fixed, 95% CI)	‐2.58 [‐6.11, 0.95]
19 Sputum dry weight (g) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

19.1 Up to 1 week (directed cough)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
19.2 Up to 1 week (PEP)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
20 Sputum volume per day (VAS score) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

20.1 Up to 2 weeks (all techniques combined)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
21 Pseudomonas density (log CFU/g) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

21.1 Up to 2 weeks (all techniques combined)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
22 Exercise capacity: 25 level modified shuttle test (m) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

22.1 Up to 2 weeks (all techniques combined)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
23 Energy (VAS score) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

23.1 Up to 2 weeks (all techniques combined)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
24 6MWT Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

24.1 Up to 3 months (PEP)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
25 Length of hospital stay (days) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

25.1 Up to 2 weeks (all techniques combined)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
26 Time to next admission (days) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

Comparison 1. NIV versus no NIV during chest physiotherapy (any technique)

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Comparison 2. NIV in overnight ventilation compared to oxygen

Outcome or subgroup title	No. of studies	No. of participants	Statistical method	Effect size
1 CFQoL chest symptom score Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

1.1 Up to 3 months	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
2 CFQoL transitional dyspnoea index Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

2.1 Up to 3 months	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
3 Symptoms of sleep‐disordered breathing Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

3.1 Epworth Sleepiness Scale (up to 3 months)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
3.2 Global PSQI (up to 3 months)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
4 Lung function during sleep Show forest plot	1		Mean Difference (Fixed, 95% CI)	Totals not selected

4.1 VI (L/m) while awake (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
4.2 VI (L/m) during REM (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
4.3 VI (L/m) during NREM (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
4.4 VT (L) while awake (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
4.5 VT (L) during REM (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
4.6 VT (L) during NREM (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
5 Respiratory rate (breaths/min) Show forest plot	1		Mean Difference (Fixed, 95% CI)	Totals not selected

5.1 RR while awake (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
5.2 RR during REM (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
5.3 RR during NREM (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
6 Lung function while awake Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

6.1 FEV1% predicted (up to 3 months)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
6.2 FVC % predicted (up to 3 months)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
7 Arterial blood gases: pH Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

7.1 Up to 3 months	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
8 Arterial blood gases: PaO₂ (mmHg) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

8.1 Up to 3 months	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
9 Arterial blood gases: PaCO₂ (mmHg) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

9.1 Up to 3 months	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
10 Arterial blood gases: HCO₃ (mmol/L) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

10.1 Up to 3 months	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
11 Arterial blood gases: SaO₂ (%) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

11.1 Up to 3 months	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
12 Exercise performance (MSWT) (metres) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

12.1 Up to 3 months	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
13 Total sleep time (min) Show forest plot	2		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

13.1 Single night	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
13.2 Up to 3 months	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
14 REM sleep architecture Show forest plot	2		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

14.1 REM (single night)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
14.2 REM %TST (single night)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
14.3 REM % TST (up to 3 months)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
15 Sleep latency (min) Show forest plot	3		Mean Difference (IV, Fixed, 95% CI)	Subtotals only

15.1 Single night	2	38	Mean Difference (IV, Fixed, 95% CI)	2.93 [‐0.32, 6.19]
15.2 Up to 3 months	1	15	Mean Difference (IV, Fixed, 95% CI)	‐5.0 [‐19.17, 9.17]
16 Nocturnal oxygen saturation (%) Show forest plot	2		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

16.1 Mean SpO2 REM (single night)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
16.2 Mean SpO2 NREM (single night)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
16.3 Mean SpO2 for TST (up to 3 months)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
16.4 TST with SpO2 < 90% (up to 3 months)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
17 Nocturnal oxygen saturation (%) Show forest plot	1		Mean Difference (Fixed, 95% CI)	Totals not selected

17.1 Percentage TST SpO2>90%	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
17.2 Percentage REM SpO2>90%	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
17.3 Percentage NREM SpO2>90%	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
18 Nocturnal TcCO₂ (mmHg Show forest plot	2		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

18.1 TcCO2 during REM (single night)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
18.2 TcCO2 during NREM (single night)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
18.3 Mean change PtCO2 (mmHg) (up to 3 months)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
18.4 Mean change PaCO2 (mmHg) (up to 3 months)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
19 Nocturnal TcCO₂ (mmHg) Show forest plot	1		Mean Difference (Fixed, 95% CI)	Totals not selected

19.1 Mean change TcCO2 NREM to REM (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
19.2 Maximum TcCO2 (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
20 Nocturnal TcCO₂ TST (mmHg) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

20.1 Mean PtCO2 TST (up to 3 months)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
20.2 Maximum PtCO2 TST (up to 3 months)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
21 Hypopneas Show forest plot	1		Relative rate (Fixed, 95% CI)	Totals not selected

21.1 Single night	1		Relative rate (Fixed, 95% CI)	0.0 [0.0, 0.0]
22 Mean heart rate (beats/min) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

22.1 Up to 3 months	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
23 Respiratory rate Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

23.1 Up to 3 months	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]

Comparison 2. NIV in overnight ventilation compared to oxygen

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Comparison 3. NIV in overnight ventilation compared to room air

Outcome or subgroup title	No. of studies	No. of participants	Statistical method	Effect size
1 CF QoL chest symptom score Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

1.1 Up to 3 months	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
2 CF QoL traditional dyspnoea index score Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

2.1 Up to 3 months	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
3 Symptoms of sleep‐disordered breathing Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

3.1 Epworth sleepiness scale (up to 3 months)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
3.2 Global PSQI (up to 3 months)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
4 Lung function during sleep Show forest plot	1		Mean Difference (Fixed, 95% CI)	Totals not selected

4.1 VI while awake (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
4.2 VI during REM (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
4.3 VI during NREM (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
4.4 VT while awake (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
4.5 VT during REM (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
4.6 VT during NREM (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
5 Respiratory rate(breaths/min) during sleep Show forest plot	1		Mean Difference (Fixed, 95% CI)	Totals not selected

5.1 RR while awake (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
5.2 RR during REM (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
5.3 RR during NREM (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
6 Mean Respiratory Rate Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

6.1 Up to 3 months	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
7 Mean Heart Rate (beats/min) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

7.1 Up to 3 months	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
8 Lung function while awake Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

8.1 FEV1% predicted (up to 3 months)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
8.2 FVC % predicted (up to 3 months)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
9 Arterial blood gases: pH Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

9.1 Up to 3 months	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
10 Arterial blood gases: PaO₂ (mmHg) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

10.1 Up to 3 months	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
11 Arterial blood gases: PaCO₂ (mmHg) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

11.1 Up to 3 months	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
12 Arterial blood gases: HCO₃ (mmol/L) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

12.1 Up to 3 months	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
13 Arterial blood gases: SaO2 (%) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

13.1 Up to 3 months	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
14 Exercise performance (metres) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

14.1 Up to 3 months	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
15 Total sleep time (min) Show forest plot	2		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

15.1 Single night	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
15.2 Up to 3 months	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
16 REM sleep architecture Show forest plot	2		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

16.1 REM (single night)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
16.2 REM %TST (single night)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
16.3 REM % TST (up to 3 months)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
17 Sleep latency Show forest plot	3		Mean Difference (IV, Fixed, 95% CI)	Subtotals only

17.1 Single night	2	38	Mean Difference (IV, Fixed, 95% CI)	‐2.63 [‐7.37, 2.11]
17.2 At 6 weeks	1	15	Mean Difference (IV, Fixed, 95% CI)	‐3.00 [‐19.88, 13.88]
18 Nocturnal oxygen saturation (%) Show forest plot	2		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

18.1 Mean SpO2 REM (single night)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
18.2 Mean SpO2 NREM (single night)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
18.3 Mean SpO2 for TST (up to 3 months)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
18.4 TST for SpO2 < 90% (up to 3 months)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
19 Nocturnal oxygen saturation (%) Show forest plot	1		Mean Difference (Fixed, 95% CI)	Totals not selected

19.1 Percentage TST SpO2 > 90% (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
19.2 Percentage REM SpO2 > 90% (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
19.3 Percentage NREM SpO2 > 90% (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
20 Nocturnal TcCO₂ (mmHg) Show forest plot	2		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

20.1 TcCO2 during REM (single night)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
20.2 TcCO2 during NREM (single night)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
20.3 Mean change PtCO2 (up to 3 months)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
20.4 Mean change PaCO2 (up to 3 months)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
21 Nocturnal TcCO₂(mmHg) Show forest plot	1		Mean Difference (Fixed, 95% CI)	Totals not selected

21.1 Mean change TcCO2 NREM to REM (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
21.2 Maximum TcCO2 (single night)	1		Mean Difference (Fixed, 95% CI)	0.0 [0.0, 0.0]
22 Nocturnal TcCO₂ TST (mmHg) Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

22.1 Mean PtCO2 TST (up to 3 months)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
22.2 Max PtCO2 TST (up to 3 months)	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]
23 Hypopneas Show forest plot	1		Relative rate (Fixed, 95% CI)	Totals not selected

23.1 Single night	1		Relative rate (Fixed, 95% CI)	0.0 [0.0, 0.0]

Comparison 3. NIV in overnight ventilation compared to room air

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Comparison 4. NIV versus no NIV during exercise testing

Outcome or subgroup title	No. of studies	No. of participants	Statistical method	Effect size
1 6 minute walk test Show forest plot	1		Mean Difference (IV, Fixed, 95% CI)	Totals not selected

1.1 Up to 1 week	1		Mean Difference (IV, Fixed, 95% CI)	0.0 [0.0, 0.0]

Comparison 4. NIV versus no NIV during exercise testing

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Referencias

References to studies included in this review

Dwyer 2015 {published data only}

Fauroux 1999 {published data only}

Gozal 1997 {published data only}

Holland 2003 {published data only}

Kofler 1998 {published data only}

Lima 2014 {published data only}

Milross 2001 {published and unpublished data}

Placidi 2006 {published data only}

Rodriguez Hortal 2016 {published data only}

Young 2008 {published data only}

References to studies excluded from this review

Elkins 2004 {published data only}

Falk 2006 {published data only}

Fauroux 2000a {published data only}

Fauroux 2000b {published data only}

Fauroux 2001 {published data only}

Fauroux 2004 {published data only}

Greenough 2004 {published data only}

Parreira 2008 {published data only}

Piper 1992 {published data only}

Regnis 1994 {published data only}

Riethmueller 2006 {published data only}

Serra 2000 {published data only}

Serra 2002 {published data only}

References to studies awaiting assessment

Petrone 2009 {published data only}

Additional references

Ballard 1996

Bott 2009

Curtin 2002

Davidson 2016

Elbourne 2002

Fuchs 1994

Higgins 2003

Hodson 1991

Jüni 2001

RevMan 2014 [Computer program]

Stata 2001 [Computer program]

UK CF Trust 2011

Yankaskas 1999

Zinman 1989

References to other published versions of this review

Moran 2003

Moran 2007

Moran 2011

Moran 2013

Characteristics of studies

Characteristics of included studies [ordered by study ID]

Characteristics of excluded studies [ordered by study ID]

Characteristics of studies awaiting assessment [ordered by study ID]

Data and analyses

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