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Table 1 Characteristics of included studies

From: The effect of respiratory muscle training on children and adolescents with cystic fibrosis: a systematic review and meta-analysis

ID

Design

Participants

Interventions

Outcomes reported

Experimental

Control

Albinni 2004 [24]

RCT (conference proceedings)

n = 16 (gender not specified)

Age range: 6–18

FEV1 > 30% pred

n = 11 (gender not specified)

Age range: 6–18

FEV1 > 30% pred

Experimental: IMT(Respifit 1000) and CET

Resistance = NR

Dosage = daily × 12 week

Setting = NR

Progression = NR

Supervision = NR

Control: CET

Lung function = FEV1 ↔ ,FVC ↔ ,MEF 50%VC↑, MEF 25%VC↑

Inspiratory muscle strength = Pimax↑

Inspiratory muscle endurance = maximal sustained ventilatory capacity↑

Maximal exercise capacity = Vo2 max↑

Timing = 0, 12 wk

PS: reduction of antibiotic use, markedly improved expectoration and reduced sensation of breathlessness

Emirza 2021 [25] (NCT03873688)

RCT

n = 15 allocated, 14 analysed (64.3% female)

Age (year):12.97 (2.76)

FEV1: 79.64 ± 27.69% pred

n = 15 allocated, 14 analysed (50% female)

Age (year):12.13 (3.44)

FEV1: 82.00 ± 22.49% pred

Experimental: RMT(respiratory muscle exercise device threshold PEP) and routine therapy and rehabilitation

Resistance = 30% of MEP ( 5-20cmH2O)

Dosage = 10 min × 2/day × 5/wk × 6 wk

Setting = NR

Progression = 2wkly MIP, MEP

Supervision = check daily exercise records

Control: shame RMT ( 5 cmH2O) + routine therapy and rehabilitation

PCF↑

Respiratory muscle strength = MIP↑, MEP↑

Lung function ↔  = FEV1, FVC, FEV1/FVC

Exercise capacity = 6MWD↑

QoL = The Turkish version of the CFQ-R (vitality, treatment, and digestive domains of QoL in the parent↑ + physical and health domains ↑)

Global rating of change score (GRoC): All patients in the groups assessed their changes as unchanged or better

Timing = 0, 6 wk (The GRoC was assessed after the training was finished)

PS: In comparing the two groups, changes in PCF and MEP were significantly higher in the training group. During the training, no adverse effect related to the study was experienced

Zeren 2019 [26] (NCT03375684)

RCT

n = 18( 50% female)

Age (year):11.66 (2.42)

Baseline of FEV1 > 70% of the predicted value

FEV1: 79.36 ± 13.67% pred

n = 18( 56% female)

Age (year):10.47 (2.03)

Baseline of FEV1 > 70% of the predicted value

FEV1: 78.69 ± 15.91% pred

Experimental: IMT( Threshold Inspiratory Muscle Trainer) + PT ( rest for at least 1 h between training)

Resistance = 30% of MIP

Dosage = 15 min × 2/day × 8 wk

Setting = hospital, home

Progression = weekly MIP

Supervision = check daily exercise records

Control: PT

Postural stability = BBS( static postural stability = PST; dynamic postural stability = LOST↑)

Lung function = FVC↑, FEV1↑, PEF↑( expressed as percentages of the predicted values)

MIP↑, MEP↑

Exercise capacity = 6MWD↑

PS: 14 patients in PT + IMT group (78%) and 15 patients in PT group (83%) completed all training sessions as planned. Adherence to the training program averaged 97.9% ± 4.2% in PT + IMT group and 97.5% ± 5.7% in PT group. In comparing the two groups, changes in MIP were significantly higher in the training group. No adverse effects were reported during the program

Sawyer 1993 [27]

RCT

n = 10( 40% female)

Age (year): 11.46(2.45)

FEV1: 89 ± 20% pred

n = 10( 50% female)

Age (year): 9.76 (2.57)

FEV1: 92 ± 29% pred

Experimental: IMT(threshold loading device)

Resistance = 60% Plmax ( Starting from -7cmH2O)

Dosage = 30 min/ day x 7 days/ wk x 10wk

Setting = home

Progression = weekly MIP

Supervision = 3 home visits per week by a nurse + diary

Control: 10% Pimax

Inspiratory muscle strength = Pimax↑

Maximal exercise testing = Exercise time on the treadmill↑

Lung function = Improvement in lung function outcomes required in systematic reviews was not reported

Bieli 2014 [28]

RCT (conference proceedings)

n = 16 (gender not specified)

Age range: 6–18

FEV1: 87.0 ± 25.8% pred

A Group: RMET( SpiroTiger®) x 8wk + chest physiotherapy x 8wk

B Group: chest physiotherapy x 8wk + RMET x 8wk

Respiratory endurance↑

Exercise endurance↑

QoL ↔ 

Lung function ↔ 

Clinical score ↔ 

Bieli 2017 [29]

RCT(two-sequence, two-period crossover design)

intervention/control( IC):

n = 11( 54.5% female)

Age (year): 15.4( 12.0; 16.6)

FEV1 > 40% pred

control/intervention( CI):

n = 11( 54.5% female)

Age (year): 13.2( 11.9; 17.8)

FEV1 > 40% pred

Intervention period: RMET by voluntary eucapnic hyperventilation( SpiroTiger®)

Resistance = The initial training conditions were defined by breathing performance representing 50% MVV

Dosage = 5–10 min × 2/day × 5/wk x 8wk

( two periods separated by a 1-week washout)

Setting = home

Progression = NR

Supervision = supervised by a physiotherapist weekly

Control period: standard chest physiotherapy

RME test: time to exhaustion↑

Exercise testing ↔ : time to exhaustion( perform on a cycle ergometer in the sitting position)

Lung function( expressed as z-scores) ↔ : FEV1, FVC, MEF 75/25

QoL: CFQ ↔ 

CFCS ↔ 

Timing = 0, 9, 17wk

  1. (1) Age is expressed in all studies as mean ± standard deviation or range; (2) ↔ not statistically signifificant changes; ↑statistically signifificant improvement
  2. Abbreviations: RCT randomized clinical trial, FEV1 forced expiratory volume in one second, IMT inspiratory muscle training, CET cycle ergometer training, MEF 50%VC mean expiratory flow at 50% of FVC, MIP (Pimax) maximal inspiratory pressure, Vo2max maximal oxygen uptake, RMT respiratory muscle training, PEP positive expiratory pressure, MEP maximal expiratory pressure, PCF peak cough flow, FVC forced vital capacity, 6MWD 6-min walking distance, CFQ-R a revised version of the Cystic Fibrosis Questionnaire, QoL quality of life, GRoC Global rating of change score, PT physical therapy, BBS Biodex Balance System, PST Postural Stability Test, LOST Limits of Stability Test, PEF peak expiratory flow, RMET respiratory muscle endurance training, MVV maximal voluntary ventilation, MEF 75/25 mean expiratory flow at 75–25% of FVC, CFCS Cystic Fibrosis clinical score, NR not reported