Findings

What do systematic reviews say?

2,261 questions · 2,280 findings · 114 reviews · Cystic Fibrosis and Genetic Disorders

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No studies found 856 · 37.5%GRADE not reported 58 · 2.5%Very low 550 · 24.1%Low 502 · 22%Moderate 271 · 11.9%High 43 · 1.9%

Avalglucosidase alfa vs alglucosidase alfa

Use of a walking aid / wheelchair · adults with LOPD

The review found no eligible studies for this question.

1 finding from 1 review

Enzyme replacement therapy for late‐onset Pompe disease 2023 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

Avalglucosidase alfa vs alglucosidase alfa

QoL · adults with LOPD

1 finding from 1 review

Enzyme replacement therapy for late‐onset Pompe disease 2023 · Cochrane Headline outcomes only
no estimate extracted
1 study · 100 participants
Low

Avalglucosidase alfa vs alglucosidase alfa

Treatment‐related AEs: total treatment‐emergent adverse events potentially related to treatment · adults with LOPD

Avalglucosidase alfa showed no clear difference in treatment‐related AEs: total treatment‐emergent adverse events… compared with alglucosidase alfa (low certainty).

1 finding from 1 review

Enzyme replacement therapy for late‐onset Pompe disease 2023 · Cochrane Headline outcomes only
RR 0.92 [0.61, 1.4]
1 study · 100 participants
Low

Alglucosidase alfa vs placebo

Use of a walking aid / wheelchair · 90 people with LOPD

The review found no eligible studies for this question.

1 finding from 1 review

Enzyme replacement therapy for late‐onset Pompe disease 2023 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

Alglucosidase alfa vs placebo

QoL (PCS in SF‐36 (measured on a scale from 0 to 100)) · 90 people with LOPD

1 finding from 1 review

Enzyme replacement therapy for late‐onset Pompe disease 2023 · Cochrane Headline outcomes only
no estimate extracted
1 study · 90 participants
Low

Alglucosidase alfa vs placebo

Treatment‐related AEs: total treatment‐emergent adverse events potentially related to treatment · 90 people with LOPD

Alglucosidase alfa showed no clear difference in treatment‐related AEs compared with placebo (low certainty).

1 finding from 1 review

Enzyme replacement therapy for late‐onset Pompe disease 2023 · Cochrane Headline outcomes only
RR 0.94 [0.64, 1.39]
1 study · 90 participants
Low

Alglucosidase alfa plus clenbuterol vs alglucosidase alfa plus placebo

Change from baseline in 6MWT (metres) · 13 people with LOPD

1 finding from 1 review

Enzyme replacement therapy for late‐onset Pompe disease 2023 · Cochrane Headline outcomes only
no estimate extracted
1 study · 10 participants
Very low

Alglucosidase alfa plus clenbuterol vs alglucosidase alfa plus placebo

FVC (% predicted) Change from baseline · 13 people with LOPD

1 finding from 1 review

Enzyme replacement therapy for late‐onset Pompe disease 2023 · Cochrane Headline outcomes only
no estimate extracted
1 study · 11 participants
Very low

Motivational interventions to improve adherence to airway clearance treatment for people with CF ‐ specifically‐composed music vs no music

Adherence · people with CF

It is very uncertain whether motivational interventions to improve adherence to airway clearance… changes adherence to treatment Morisky‐Green questionnaire (number of… compared with no music (very low certainty).

1 finding from 1 review

Interventions for improving adherence to airway clearance treatment and exercise in people with cystic fibrosis 2023 · Cochrane Headline outcomes only
RR 1.56 [1.01, 2.4]
1 study · 30 participants
Very low

Motivational interventions to improve adherence to airway clearance treatment for people with CF ‐ self‐selected music vs no music

Frequency of non‐hospitalised respiratory infection · people with CF

The review found no eligible studies for this question.

1 finding from 1 review

Interventions for improving adherence to airway clearance treatment and exercise in people with cystic fibrosis 2023 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

Motivational interventions to improve adherence to airway clearance treatment for people with CF ‐ self‐selected music vs no music

FEV1 % predicted: absolute change from baseline · people with CF

The review found no eligible studies for this question.

1 finding from 1 review

Interventions for improving adherence to airway clearance treatment and exercise in people with cystic fibrosis 2023 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

Motivational interventions to improve adherence to airway clearance treatment for people with CF ‐ self‐selected music vs no music

FVC (% predicted) Change from baseline · people with CF

The review found no eligible studies for this question.

1 finding from 1 review

Interventions for improving adherence to airway clearance treatment and exercise in people with cystic fibrosis 2023 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

Motivational interventions to improve adherence to airway clearance treatment for people with CF ‐ self‐selected music vs no music

QoL · people with CF

The review found no eligible studies for this question.

1 finding from 1 review

Interventions for improving adherence to airway clearance treatment and exercise in people with cystic fibrosis 2023 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

Motivational interventions to improve adherence to airway clearance treatment for people with CF ‐ self‐selected music vs no music

Exercise capacity (change from baseline) · people with CF

The review found no eligible studies for this question.

1 finding from 1 review

Interventions for improving adherence to airway clearance treatment and exercise in people with cystic fibrosis 2023 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

Motivational interventions to improve adherence to airway clearance treatment for people with CF ‐ specifically‐composed music vs self‐selected music

Adherence · people with CF

1 finding from 1 review

Interventions for improving adherence to airway clearance treatment and exercise in people with cystic fibrosis 2023 · Cochrane Headline outcomes only
RR 1.52 [0.97, 2.38]
1 study · 28 participants
Very low

Motivational interventions to improve adherence to airway clearance treatment for people with CF ‐ specifically‐composed music vs self‐selected music

Frequency of hospitalisation for respiratory infection · people with CF

It is very uncertain whether motivational interventions to improve adherence to airway clearance… changes frequency of hospitalisation for respiratory infection compared with self‐selected music (very low certainty).

1 finding from 1 review

Interventions for improving adherence to airway clearance treatment and exercise in people with cystic fibrosis 2023 · Cochrane Headline outcomes only
RR 0.29 [0.03, 2.45]
1 study · 28 participants
Very low

Motivational interventions to improve adherence to airway clearance treatment for people with CF ‐ specifically‐composed music vs self‐selected music

Frequency of non‐hospitalised respiratory infection · people with CF

The review found no eligible studies for this question.

1 finding from 1 review

Interventions for improving adherence to airway clearance treatment and exercise in people with cystic fibrosis 2023 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

Motivational interventions to improve adherence to airway clearance treatment for people with CF ‐ specifically‐composed music vs self‐selected music

FEV1 % predicted: absolute change from baseline · people with CF

The review found no eligible studies for this question.

1 finding from 1 review

Interventions for improving adherence to airway clearance treatment and exercise in people with cystic fibrosis 2023 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

Motivational interventions to improve adherence to airway clearance treatment for people with CF ‐ specifically‐composed music vs self‐selected music

FVC (% predicted) Change from baseline · people with CF

The review found no eligible studies for this question.

1 finding from 1 review

Interventions for improving adherence to airway clearance treatment and exercise in people with cystic fibrosis 2023 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

Motivational interventions to improve adherence to airway clearance treatment for people with CF ‐ specifically‐composed music vs no music

Frequency of hospitalisation for respiratory infection · people with CF

1 finding from 1 review

Interventions for improving adherence to airway clearance treatment and exercise in people with cystic fibrosis 2023 · Cochrane Headline outcomes only
RR 0.17 [0.02, 1.22]
1 study · 30 participants
Very low