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Search review questions

Each row is one question a systematic review asked: who, what, compared with what, and which outcome, with the effect it found and how certain that is. Words match anywhere in the field.

Limit to evidence status or GRADE (optional)

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

current versions
No studies 856 · 37.5%Ungraded 58 · 2.5%Very low 550 · 24.1%Low 502 · 22%Moderate 271 · 11.9%High 43 · 1.9%

Ungraded means an effect was reported without a GRADE rating; it is not a gap. No studies means the review found nothing to answer the question. The six categories are a display order, not a score.

QuestionEffectEvidenceStatus / GRADE
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
Interventions for improving adherence to airway clearance treatment and exercise in people with cystic… (2023) · Cochrane
RR 0.17 [0.02, 1.22]1 study · 30 participantsVery low
Motivational interventions to improve adherence to airway clearance treatment for people with CF ‐ specifically‐composed music vs self‐selected music
QoL (change from baseline) · people with CF
Interventions for improving adherence to airway clearance treatment and exercise in people with cystic… (2023) · Cochrane
no estimate extractedNRNo studies
Motivational interventions to improve adherence to airway clearance treatment for people with CF ‐ specifically‐composed music vs self‐selected music
Exercise capacity (change from baseline) · people with CF
Interventions for improving adherence to airway clearance treatment and exercise in people with cystic… (2023) · Cochrane
no estimate extractedNRNo studies
Intervention NR
Adherence to treatment (number of participants undertaking physical activity) · people with CF
Interventions for improving adherence to airway clearance treatment and exercise in people with cystic… (2023) · Cochrane
RR 3.67 [1.24, 10.85]1 study · 34 participantsVery low
Intervention NR
Frequency of hospitalisation for respiratory infection · people with CF
Interventions for improving adherence to airway clearance treatment and exercise in people with cystic… (2023) · Cochrane
no estimate extractedNRNo studies
Intervention NR
Frequency of non‐hospitalised respiratory infection · people with CF
Interventions for improving adherence to airway clearance treatment and exercise in people with cystic… (2023) · Cochrane
no estimate extractedNRNo studies
Intervention NR
FEV1 % predicted (change from baseline) · people with CF
Interventions for improving adherence to airway clearance treatment and exercise in people with cystic… (2023) · Cochrane
no estimate extracted1 study · 34 participantsVery low
Intervention NR
FVC % predicted (change from baseline) · people with CF
Interventions for improving adherence to airway clearance treatment and exercise in people with cystic… (2023) · Cochrane
no estimate extracted1 study · 34 participantsVery low
Intervention NR
QoL (change from baseline) · people with CF
Interventions for improving adherence to airway clearance treatment and exercise in people with cystic… (2023) · Cochrane
no estimate extracted1 study · 34 participantsVery low
Intervention NR
Exercise capacity (change from baseline) VO2 peak · people with CF
Interventions for improving adherence to airway clearance treatment and exercise in people with cystic… (2023) · Cochrane
no estimate extracted1 study · 34 participantsVery low
Motivational interventions to improve adherence to airway clearance treatment for people with CF ‐ specifically‐composed music vs no music
Frequency of non‐hospitalised respiratory infection · people with CF
Interventions for improving adherence to airway clearance treatment and exercise in people with cystic… (2023) · Cochrane
no estimate extractedNRNo studies
Motivational interventions to improve adherence to airway clearance treatment for people with CF ‐ specifically‐composed music vs no music
FEV1 % predicted (change from baseline) · people with CF
Interventions for improving adherence to airway clearance treatment and exercise in people with cystic… (2023) · Cochrane
no estimate extractedNRNo studies
Motivational interventions to improve adherence to airway clearance treatment for people with CF ‐ specifically‐composed music vs no music
FVC % predicted (change from baseline) · people with CF
Interventions for improving adherence to airway clearance treatment and exercise in people with cystic… (2023) · Cochrane
no estimate extractedNRNo studies
Motivational interventions to improve adherence to airway clearance treatment for people with CF ‐ specifically‐composed music vs no music
QoL (change from baseline) · people with CF
Interventions for improving adherence to airway clearance treatment and exercise in people with cystic… (2023) · Cochrane
no estimate extractedNRNo studies
Motivational interventions to improve adherence to airway clearance treatment for people with CF ‐ specifically‐composed music vs no music
Exercise capacity (change from baseline) · people with CF
Interventions for improving adherence to airway clearance treatment and exercise in people with cystic… (2023) · Cochrane
no estimate extractedNRNo studies
Motivational interventions to improve adherence to airway clearance treatment for people with CF ‐ self‐selected music vs no music
Adherence to treatment Morisky‐Green questionnaire (number of participants showing full adherence) · people with CF
Interventions for improving adherence to airway clearance treatment and exercise in people with cystic… (2023) · Cochrane
RR 1.03 [0.57, 1.86]1 study · 28 participantsVery low
Motivational interventions to improve adherence to airway clearance treatment for people with CF ‐ self‐selected music vs no music
Frequency of hospitalisation for respiratory infection · people with CF
Interventions for improving adherence to airway clearance treatment and exercise in people with cystic… (2023) · Cochrane
RR 0.58 [0.18, 1.86]1 study · 28 participantsVery low
Digital technology (TIM) vs standard treatment (TBM)
Adherence to the inhaled treatment: · children aged 5 to 16 years
Digital technology for monitoring adherence to inhaled therapies in people with cystic fibrosis (2023) · Cochrane
MD 24 [2.95, 45.05]1 study · 20 participantsLow
Digital technology (web‐based platform CFHealthHub) vs usual care
Number of pulmonary exacerbations · people with CF aged 16 years and over
Digital technology for monitoring adherence to inhaled therapies in people with cystic fibrosis (2023) · Cochrane
rate ratio 0.921 study · 607 participantsModerate
Digital technology (TIM) vs standard treatment (TBM)
Treatment burden · children aged 5 to 16 years
Digital technology for monitoring adherence to inhaled therapies in people with cystic fibrosis (2023) · Cochrane
no estimate extractedNRNo studies
Digital technology (TIM) vs standard treatment (TBM)
QoL · children aged 5 to 16 years
Digital technology for monitoring adherence to inhaled therapies in people with cystic fibrosis (2023) · Cochrane
no estimate extractedNRNo studies
Digital technology (TIM) vs standard treatment (TBM)
FEV1 (mean change from baseline, % predicted) · children aged 5 to 16 years
Digital technology for monitoring adherence to inhaled therapies in people with cystic fibrosis (2023) · Cochrane
MD 11 study · 20 participantsLow
Digital technology (TIM) vs standard treatment (TBM)
Number of pulmonary exacerbations · children aged 5 to 16 years
Digital technology for monitoring adherence to inhaled therapies in people with cystic fibrosis (2023) · Cochrane
no estimate extractedNRNo studies
Digital technology (web‐based platform CFHealthHub) vs usual care
Adherence to the inhaled treatment: percentage adherence · people with CF aged 16 years and over
Digital technology for monitoring adherence to inhaled therapies in people with cystic fibrosis (2023) · Cochrane
MD 18 [12.9, 23.1]1 study · 588 participantsModerate
Digital technology (web‐based platform CFHealthHub) vs usual care
Treatment burden: CFQ‐R treatment burden domain score (higher score is better) · people with CF aged 16 years and over
Digital technology for monitoring adherence to inhaled therapies in people with cystic fibrosis (2023) · Cochrane
MD 5.1 [1.79, 8.41]1 study · 539 participantsModerate
Digital technology (web‐based platform CFHealthHub) vs usual care
QoL: CFQ‐R domain scores at end of study · people with CF aged 16 years and over
Digital technology for monitoring adherence to inhaled therapies in people with cystic fibrosis (2023) · Cochrane
MD 3.21 study · 538 participantsModerate
Digital technology (web‐based platform CFHealthHub) vs usual care
FEV1: mean FEV1 % predicted at end of study · people with CF aged 16 years and over
Digital technology for monitoring adherence to inhaled therapies in people with cystic fibrosis (2023) · Cochrane
MD 3.71 study · 556 participantsLow
Summary of findings: psychological interventions vs usual care
Adherence to inhaled therapies: % of prescribed inhaled therapies taken · children, adolescents or adults with CF
Psychological interventions for improving adherence to inhaled therapies in people with cystic fibrosis (2023) · Cochrane
MD 9.5 [8.6, 10.4]1 study · 588 participantsModerate
Summary of findings: psychological interventions vs an active comparator
QoL CFQ‐R (Quittner 2009) · adults (aged 16 years and over) with CF
Psychological interventions for improving adherence to inhaled therapies in people with cystic fibrosis (2023) · Cochrane
no estimate extracted1 study · 95 participantsVery low
Summary of findings: psychological interventions vs an active comparator
Lung function FEV1 % predicted · adults (aged 16 years and over) with CF
Psychological interventions for improving adherence to inhaled therapies in people with cystic fibrosis (2023) · Cochrane
no estimate extracted1 study · 125 participantsVery low
Summary of findings: psychological interventions vs an active comparator
Pulmonary exacerbations · adults (aged 16 years and over) with CF
Psychological interventions for improving adherence to inhaled therapies in people with cystic fibrosis (2023) · Cochrane
no estimate extracted1 study · 128 participantsVery low
Summary of findings: psychological interventions vs usual care
Treatment‐related adverse events Anxiety: GAD‐7 (Spitzer 2006) · children, adolescents or adults with CF
Psychological interventions for improving adherence to inhaled therapies in people with cystic fibrosis (2023) · Cochrane
MD 0.31 study · 535 participantsLow
Summary of findings: psychological interventions vs usual care
Treatment‐related adverse events Depression PHQ‐8 (Kroenke 2009) · children, adolescents or adults with CF
Psychological interventions for improving adherence to inhaled therapies in people with cystic fibrosis (2023) · Cochrane
no estimate extracted1 study · 534 participantsLow
Summary of findings: psychological interventions vs usual care
QoL CFQ‐R (Quittner 2009) · children, adolescents or adults with CF
Psychological interventions for improving adherence to inhaled therapies in people with cystic fibrosis (2023) · Cochrane
MD 3.9 [1.2, 6.6]1 study · 539 participantsLow
Summary of findings: psychological interventions vs usual care
Lung function FEV1 % predicted · children, adolescents or adults with CF
Psychological interventions for improving adherence to inhaled therapies in people with cystic fibrosis (2023) · Cochrane
MD 1.41 study · 556 participantsModerate
Summary of findings: psychological interventions vs usual care
Pulmonary exacerbations · children, adolescents or adults with CF
Psychological interventions for improving adherence to inhaled therapies in people with cystic fibrosis (2023) · Cochrane
no estimate extracted1 study · 607 participantsModerate
Summary of findings: psychological interventions vs an active comparator
Adherence to inhaled therapies cMPR · adults (aged 16 years and over) with CF
Psychological interventions for improving adherence to inhaled therapies in people with cystic fibrosis (2023) · Cochrane
no estimate extracted1 study · 95 participantsVery low
Summary of findings: psychological interventions vs an active comparator
Treatment‐related adverse events Anxiety · adults (aged 16 years and over) with CF
Psychological interventions for improving adherence to inhaled therapies in people with cystic fibrosis (2023) · Cochrane
no estimate extractedNRNo studies
Summary of findings: psychological interventions vs an active comparator
Treatment‐related adverse events Depression · adults (aged 16 years and over) with CF
Psychological interventions for improving adherence to inhaled therapies in people with cystic fibrosis (2023) · Cochrane
no estimate extractedNRNo studies
Single foetal haemoglobin inducer at any dose or duration vs usual care or placebo
Frequency of transfusion (transfused volume/year) · people with non‐transfusion‐dependent β‐thalassaemia
Foetal haemoglobin inducers for reducing blood transfusion in non‐transfusion‐dependent beta‐thalassaemias (2023) · Cochrane
no estimate extractedNRNo studies
Single foetal haemoglobin inducer at any dose or duration vs another foetal haemoglobin inducer
HbF (%) · people with non‐transfusion‐dependent β‐thalassaemia
Foetal haemoglobin inducers for reducing blood transfusion in non‐transfusion‐dependent beta‐thalassaemias (2023) · Cochrane
no estimate extracted3 studies · 84 participantsLow
Single foetal haemoglobin inducer at any dose or duration vs another foetal haemoglobin inducer
QoL · people with non‐transfusion‐dependent β‐thalassaemia
Foetal haemoglobin inducers for reducing blood transfusion in non‐transfusion‐dependent beta‐thalassaemias (2023) · Cochrane
no estimate extractedNRNo studies
Single foetal haemoglobin inducer at any dose or duration vs another foetal haemoglobin inducer
Adverse events · people with non‐transfusion‐dependent β‐thalassaemia
Foetal haemoglobin inducers for reducing blood transfusion in non‐transfusion‐dependent beta‐thalassaemias (2023) · Cochrane
no estimate extracted4 studies · 118 participantsVery low
One dose or dose regimen of a foetal haemoglobin inducer vs another dose or dose regimen of the same foetal haemoglobin inducer
Frequency of transfusion (transfused volume/ year) · people with non‐transfusion‐dependent β‐thalassaemia
Foetal haemoglobin inducers for reducing blood transfusion in non‐transfusion‐dependent beta‐thalassaemias (2023) · Cochrane
no estimate extractedNRNo studies
One dose or dose regimen of a foetal haemoglobin inducer vs another dose or dose regimen of the same foetal haemoglobin inducer
Transfusion‐free interval (days) · people with non‐transfusion‐dependent β‐thalassaemia
Foetal haemoglobin inducers for reducing blood transfusion in non‐transfusion‐dependent beta‐thalassaemias (2023) · Cochrane
no estimate extractedNRNo studies
One dose or dose regimen of a foetal haemoglobin inducer vs another dose or dose regimen of the same foetal haemoglobin inducer
Haemoglobin (g/dL) · people with non‐transfusion‐dependent β‐thalassaemia
Foetal haemoglobin inducers for reducing blood transfusion in non‐transfusion‐dependent beta‐thalassaemias (2023) · Cochrane
no estimate extracted1 study · 61 participantsVery low
One dose or dose regimen of a foetal haemoglobin inducer vs another dose or dose regimen of the same foetal haemoglobin inducer
HbF (%) · people with non‐transfusion‐dependent β‐thalassaemia
Foetal haemoglobin inducers for reducing blood transfusion in non‐transfusion‐dependent beta‐thalassaemias (2023) · Cochrane
no estimate extracted1 study · 61 participantsVery low
One dose or dose regimen of a foetal haemoglobin inducer vs another dose or dose regimen of the same foetal haemoglobin inducer
QoL · people with non‐transfusion‐dependent β‐thalassaemia
Foetal haemoglobin inducers for reducing blood transfusion in non‐transfusion‐dependent beta‐thalassaemias (2023) · Cochrane
no estimate extractedNRNo studies
One dose or dose regimen of a foetal haemoglobin inducer vs another dose or dose regimen of the same foetal haemoglobin inducer
Adverse events · people with non‐transfusion‐dependent β‐thalassaemia
Foetal haemoglobin inducers for reducing blood transfusion in non‐transfusion‐dependent beta‐thalassaemias (2023) · Cochrane
no estimate extracted2 studies · 82 participantsVery low
One dose or dose regimen of a foetal haemoglobin inducer vs another dose or dose regimen of the same foetal haemoglobin inducer
Neutropenia (WBC < 1 × 103/μL, ANC < 0.5 × 103/μL) · people with non‐transfusion‐dependent β‐thalassaemia
Foetal haemoglobin inducers for reducing blood transfusion in non‐transfusion‐dependent beta‐thalassaemias (2023) · Cochrane
RR 9.93 [1.34, 73.67]1 study · 61 participantsVery low