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Comparison 1 RMT (80% of maximal effort) versus control, Outcome 1 Forced expiratory volume at one second (L).
Figuras y tablas -
Analysis 1.1

Comparison 1 RMT (80% of maximal effort) versus control, Outcome 1 Forced expiratory volume at one second (L).

Comparison 1 RMT (80% of maximal effort) versus control, Outcome 2 Forced vital capacity (L).
Figuras y tablas -
Analysis 1.2

Comparison 1 RMT (80% of maximal effort) versus control, Outcome 2 Forced vital capacity (L).

Comparison 1 RMT (80% of maximal effort) versus control, Outcome 3 Chronic Respiratory Disease Questionnaire (mastery).
Figuras y tablas -
Analysis 1.3

Comparison 1 RMT (80% of maximal effort) versus control, Outcome 3 Chronic Respiratory Disease Questionnaire (mastery).

Comparison 1 RMT (80% of maximal effort) versus control, Outcome 4 Chronic Respiratory Disease Questionnaire (emotion).
Figuras y tablas -
Analysis 1.4

Comparison 1 RMT (80% of maximal effort) versus control, Outcome 4 Chronic Respiratory Disease Questionnaire (emotion).

Comparison 2 RMT (60% of maximal effort) versus control, Outcome 1 Forced expiratory volume at one second (L).
Figuras y tablas -
Analysis 2.1

Comparison 2 RMT (60% of maximal effort) versus control, Outcome 1 Forced expiratory volume at one second (L).

Comparison 2 RMT (60% of maximal effort) versus control, Outcome 2 PImax (cm H₂O).
Figuras y tablas -
Analysis 2.2

Comparison 2 RMT (60% of maximal effort) versus control, Outcome 2 PImax (cm H₂O).

Comparison 3 RMT (40% of maximal effort) versus control, Outcome 1 Forced expiratory volume at one second (L).
Figuras y tablas -
Analysis 3.1

Comparison 3 RMT (40% of maximal effort) versus control, Outcome 1 Forced expiratory volume at one second (L).

Comparison 3 RMT (40% of maximal effort) versus control, Outcome 2 Forced expiratory volume at one second (% predicted).
Figuras y tablas -
Analysis 3.2

Comparison 3 RMT (40% of maximal effort) versus control, Outcome 2 Forced expiratory volume at one second (% predicted).

Comparison 3 RMT (40% of maximal effort) versus control, Outcome 3 Forced vital capacity (L).
Figuras y tablas -
Analysis 3.3

Comparison 3 RMT (40% of maximal effort) versus control, Outcome 3 Forced vital capacity (L).

Comparison 3 RMT (40% of maximal effort) versus control, Outcome 4 Forced vital capacity (% predicted).
Figuras y tablas -
Analysis 3.4

Comparison 3 RMT (40% of maximal effort) versus control, Outcome 4 Forced vital capacity (% predicted).

Comparison 3 RMT (40% of maximal effort) versus control, Outcome 5 Inspiratory muscle endurance (% PImax).
Figuras y tablas -
Analysis 3.5

Comparison 3 RMT (40% of maximal effort) versus control, Outcome 5 Inspiratory muscle endurance (% PImax).

Comparison 4 RMT (20% of maximal effort) versus control, Outcome 1 Forced expiratory volume at one second (L).
Figuras y tablas -
Analysis 4.1

Comparison 4 RMT (20% of maximal effort) versus control, Outcome 1 Forced expiratory volume at one second (L).

Comparison 4 RMT (20% of maximal effort) versus control, Outcome 2 Forced vital capacity (L).
Figuras y tablas -
Analysis 4.2

Comparison 4 RMT (20% of maximal effort) versus control, Outcome 2 Forced vital capacity (L).

Respiratory muscle training compared with control for cystic fibrosis

Patient or population: adults and children with cystic fibrosis

Settings: outpatients

Intervention: respiratory muscle trainingₑ

Comparison: controlₑ

Outcomes

Illustrative comparative risks* (95% CI)

Relative effect
(95% CI)

No of participants
(studies)

Quality of the evidence
(GRADE)

Comments

Assumed risk

Corresponding risk

Controlₑ

Respiratory muscle trainingₑ

FEV1: % predicted

Follow‐up: 6‐12 weeks

No significant differences between the respiratory muscle training group and the control group were reported in any study.

NA

145

(7 studies including 2 cross‐over studies)

⊕⊝⊝⊝
very low2,3

Studies reported FEV1 as % predicted, litres or z score.

One study with respiratory muscle training level 30% of maximal effort reported a significant improvement within the training group.

FVC: % predicted

Follow‐up: 6‐12 weeks

No significant differences between the respiratory muscle training group and the control group were reported in any study.

NA

114

(5 studies including 1 cross‐over study)

⊕⊝⊝⊝
very low2,3

Studies reported FVC as % predicted, litres or z score.

One study with respiratory muscle training level 30% of maximal effort reported a significant improvement within the training group.

Exercise capacity: VO2max (mL/kg/min)

Follow‐up: 6‐12 weeks

No significant differences between the respiratory muscle training group and the control group were reported in any study.

NA

54

(3 studies including 1 cross‐over study)

⊕⊝⊝⊝
very low2,3

One study with an unspecified level of resistance reported a significant improvement within the respiratory muscle training group.

HRQoL: total score

Follow‐up: 8 weeks

Two studies reported no significant differences between the respiratory muscle training group and the control group.

One study reported significant improvements in the parameters of mastery and emotion in the respiratory muscle training group compared to the control group.

NA

69

(3 studies including 1 cross‐over study)

⊕⊝⊝⊝
very low2,3

Two studies used the Chronic Respiratory Disease Questionnaire (CRDQ) and one study used the cystic fibrosis questionnaire (CFQ).

Respiratory muscle function: maximal inspiratory pressure (PImax)

Follow‐up: 6‐10 weeks

Significant improvements were observed in all respiratory muscle training groups.

Two studies reported no significant differences between the respiratory muscle training group and the control group.

NA

51

(3 studies including 1 cross‐over study)

⊕⊕⊝⊝
low2

Respiratory muscle function: inspiratory capacity

Follow‐up: NA

NA

*The basis for the assumed risk (e.g. the median control group risk across studies) is provided in footnotes. The corresponding risk (and its 95% confidence interval) is based on the assumed risk in the comparison group and the relative effect of the intervention (and its 95% CI).
CI: confidence interval; FEV1: forced expiratory volume in 1 second; FVC: forced vital capacity; HRQoL: health related quality of life;NA: not applicable; VO₂max: maximal oxygen uptake.

GRADE Working Group grades of evidence
High quality: Further research is very unlikely to change our confidence in the estimate of effect.
Moderate quality: Further research is likely to have an important impact on our confidence in the estimate of effect and may change the estimate.
Low quality: Further research is very likely to have an important impact on our confidence in the estimate of effect and is likely to change the estimate.
Very low quality: We are very uncertain about the estimate.

1. The resistance level of the respiratory muscle training intervention was variable; three studies used 80% of maximal effort, one study used 60% of maximal effort, one study used 40% of maximal effort, one study used 30% of maximal effort and three studies did not specify the level of resistance. Control groups were also variable; cycle ergometer, H20, treatment as usual, standard chest physiotherapy, low resistance threshold loading device, no training or sham training.

2. Downgraded twice due to serious risk of bias: the included studies lacked methodological detail relating to methods of randomisation, allocation concealment and blinding. Most of the studies were at high risk of bias due to lack of blinding, incomplete outcome data or selective reporting, or both.

3. Downgraded due to imprecision: studies included a small number of participants and numerical results were not available for some of the studies.

Figuras y tablas -
Comparison 1. RMT (80% of maximal effort) versus control

Outcome or subgroup title

No. of studies

No. of participants

Statistical method

Effect size

1 Forced expiratory volume at one second (L) Show forest plot

1

Mean Difference (IV, Fixed, 95% CI)

Totals not selected

1.1 Two to six months

1

Mean Difference (IV, Fixed, 95% CI)

0.0 [0.0, 0.0]

2 Forced vital capacity (L) Show forest plot

1

Mean Difference (IV, Fixed, 95% CI)

Totals not selected

2.1 Two to six months

1

Mean Difference (IV, Fixed, 95% CI)

0.0 [0.0, 0.0]

3 Chronic Respiratory Disease Questionnaire (mastery) Show forest plot

1

Mean Difference (IV, Fixed, 95% CI)

Totals not selected

4 Chronic Respiratory Disease Questionnaire (emotion) Show forest plot

1

Mean Difference (IV, Fixed, 95% CI)

Totals not selected

Figuras y tablas -
Comparison 1. RMT (80% of maximal effort) versus control
Comparison 2. RMT (60% of maximal effort) versus control

Outcome or subgroup title

No. of studies

No. of participants

Statistical method

Effect size

1 Forced expiratory volume at one second (L) Show forest plot

1

Mean Difference (IV, Fixed, 95% CI)

Totals not selected

1.1 Two to six months

1

Mean Difference (IV, Fixed, 95% CI)

0.0 [0.0, 0.0]

2 PImax (cm H₂O) Show forest plot

1

Mean Difference (IV, Fixed, 95% CI)

Totals not selected

2.1 Two to six months

1

Mean Difference (IV, Fixed, 95% CI)

0.0 [0.0, 0.0]

Figuras y tablas -
Comparison 2. RMT (60% of maximal effort) versus control
Comparison 3. RMT (40% of maximal effort) versus control

Outcome or subgroup title

No. of studies

No. of participants

Statistical method

Effect size

1 Forced expiratory volume at one second (L) Show forest plot

1

Mean Difference (IV, Fixed, 95% CI)

Totals not selected

1.1 Less than two months

1

Mean Difference (IV, Fixed, 95% CI)

0.0 [0.0, 0.0]

2 Forced expiratory volume at one second (% predicted) Show forest plot

1

Mean Difference (IV, Fixed, 95% CI)

Totals not selected

2.1 Less than two months

1

Mean Difference (IV, Fixed, 95% CI)

0.0 [0.0, 0.0]

3 Forced vital capacity (L) Show forest plot

1

Mean Difference (IV, Fixed, 95% CI)

Totals not selected

3.1 Less than two months

1

Mean Difference (IV, Fixed, 95% CI)

0.0 [0.0, 0.0]

4 Forced vital capacity (% predicted) Show forest plot

1

Mean Difference (IV, Fixed, 95% CI)

Totals not selected

4.1 Less than two months

1

Mean Difference (IV, Fixed, 95% CI)

0.0 [0.0, 0.0]

5 Inspiratory muscle endurance (% PImax) Show forest plot

1

Mean Difference (IV, Fixed, 95% CI)

Totals not selected

5.1 Less than two months

1

Mean Difference (IV, Fixed, 95% CI)

0.0 [0.0, 0.0]

Figuras y tablas -
Comparison 3. RMT (40% of maximal effort) versus control
Comparison 4. RMT (20% of maximal effort) versus control

Outcome or subgroup title

No. of studies

No. of participants

Statistical method

Effect size

1 Forced expiratory volume at one second (L) Show forest plot

1

Mean Difference (IV, Fixed, 95% CI)

Totals not selected

1.1 Two to six months

1

Mean Difference (IV, Fixed, 95% CI)

0.0 [0.0, 0.0]

2 Forced vital capacity (L) Show forest plot

1

Mean Difference (IV, Fixed, 95% CI)

Totals not selected

2.1 Two to six months

1

Mean Difference (IV, Fixed, 95% CI)

0.0 [0.0, 0.0]

Figuras y tablas -
Comparison 4. RMT (20% of maximal effort) versus control