Findings

What do systematic reviews say?

873 questions · 883 findings · 38 reviews · Cystic Fibrosis and Genetic Disorders

Sort:Best matchNewest Highest certainty Download CSV

No studies found 319 · 36.1%GRADE not reported 18 · 2%Very low 198 · 22.4%Low 186 · 21.1%Moderate 147 · 16.6%High 15 · 1.7%

Summary of findings ‐ monotherapy: lumacaftor vs placebo

Adverse events · adults and children with cystic fibrosis

1 finding from 1 review

Corrector therapies (with or without potentiators) for people with cystic fibrosis with class II CFTR gene variants… 2023 · Cochrane Headline outcomes only
no estimate extracted
2 studies · 115 participants
Very low

Summary of findings ‐ monotherapy: lumacaftor vs placebo

Time to first pulmonary exacerbation · adults and children with cystic fibrosis

The review found no eligible studies for this question.

1 finding from 1 review

Corrector therapies (with or without potentiators) for people with cystic fibrosis with class II CFTR gene variants… 2023 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

Summary of findings ‐ 400 mg inhaled mannitol vs 50 mg inhaled mannitol

HRQoL ‐ all domains (change from baseline) Scale: age‐appropriate versions of the CFQ‐R questionnaire · adults, children and young people with CF

1 finding from 1 review

Inhaled mannitol for cystic fibrosis 2020 · Cochrane Headline outcomes only
no estimate extracted
2 studies · 324 participants
Low

Summary of findings ‐ 400 mg inhaled mannitol vs 50 mg inhaled mannitol

Lung function: FEV1 mL (change from baseline) · adults, children and young people with CF

1 finding from 1 review

Inhaled mannitol for cystic fibrosis 2020 · Cochrane Headline outcomes only
no estimate extracted
2 studies · 600 participants
Moderate

Summary of findings ‐ 400 mg inhaled mannitol vs 50 mg inhaled mannitol

FEV1 % predicted: absolute change from baseline · adults, children and young people with CF

1 finding from 1 review

Inhaled mannitol for cystic fibrosis 2020 · Cochrane Headline outcomes only
no estimate extracted
2 studies · 600 participants
Moderate

Summary of findings ‐ 400 mg inhaled mannitol vs 50 mg inhaled mannitol

FVC · adults, children and young people with CF

1 finding from 1 review

Inhaled mannitol for cystic fibrosis 2020 · Cochrane Headline outcomes only
no estimate extracted
2 studies · 600 participants
Moderate

Summary of findings ‐ 400 mg inhaled mannitol vs 50 mg inhaled mannitol

Lung function: FEF25‐75 mL/s (change from baseline) · adults, children and young people with CF

1 finding from 1 review

Inhaled mannitol for cystic fibrosis 2020 · Cochrane Headline outcomes only
no estimate extracted
2 studies · 600 participants
Moderate

Summary of findings ‐ 400 mg inhaled mannitol vs 50 mg inhaled mannitol

Adverse events relating to treatment Scale: mild, moderate, severe and total · adults, children and young people with CF

1 finding from 1 review

Inhaled mannitol for cystic fibrosis 2020 · Cochrane Headline outcomes only
no estimate extracted
2 studies · 600 participants
Moderate

Summary of findings ‐ monotherapy: cavosonstat vs placebo

Quality of life: CFQ‐R respiratory domain: absolute change from baseline · adults and children with cystic fibrosis

1 finding from 1 review

Corrector therapies (with or without potentiators) for people with cystic fibrosis with class II CFTR gene variants… 2023 · Cochrane Headline outcomes only
no estimate extracted
1 study · 24 participants
Very low

Summary of findings ‐ monotherapy: cavosonstat vs placebo

FEV1 % predicted: relative change from baseline · adults and children with cystic fibrosis

The review found no eligible studies for this question.

1 finding from 1 review

Corrector therapies (with or without potentiators) for people with cystic fibrosis with class II CFTR gene variants… 2023 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

Summary of findings ‐ monotherapy: cavosonstat vs placebo

FEV1 % predicted: absolute change from baseline · adults and children with cystic fibrosis

1 finding from 1 review

Corrector therapies (with or without potentiators) for people with cystic fibrosis with class II CFTR gene variants… 2023 · Cochrane Headline outcomes only
no estimate extracted
1 study · 26 participants
Very low

Summary of findings ‐ monotherapy: cavosonstat vs placebo

Adverse events: occurring in at least 10% of cavosonstat treated participants · adults and children with cystic fibrosis

1 finding from 1 review

Corrector therapies (with or without potentiators) for people with cystic fibrosis with class II CFTR gene variants… 2023 · Cochrane Headline outcomes only
no estimate extracted
1 study · 26 participants
Very low

Summary of findings ‐ monotherapy: cavosonstat vs placebo

Time to first pulmonary exacerbation · adults and children with cystic fibrosis

The review found no eligible studies for this question.

1 finding from 1 review

Corrector therapies (with or without potentiators) for people with cystic fibrosis with class II CFTR gene variants… 2023 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

Summary of findings ‐ monotherapy: cavosonstat vs placebo

Survival · adults and children with cystic fibrosis

1 finding from 1 review

Corrector therapies (with or without potentiators) for people with cystic fibrosis with class II CFTR gene variants… 2023 · Cochrane Headline outcomes only
no estimate extracted
1 study · 26 participants
Very low

Summary of findings ‐ monotherapy: cavosonstat vs placebo

Quality of life: total score · adults and children with cystic fibrosis

The review found no eligible studies for this question.

1 finding from 1 review

Corrector therapies (with or without potentiators) for people with cystic fibrosis with class II CFTR gene variants… 2023 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

Psychological interventions vs usual care

Adherence to inhaled therapies cMPR · children, adolescents or adults with CF

Summary of findings: psychological interventions probably leads to higher adherence to inhaled therapies: % of prescribed inhaled therapies… compared with usual care (moderate certainty).

1 finding from 1 review

Psychological interventions for improving adherence to inhaled therapies in people with cystic fibrosis 2023 · Cochrane Headline outcomes only
MD 9.5 [8.6, 10.4]
1 study · 588 participants
Moderate

Psychological interventions vs active comparators

QoL · adults (aged 16 years and over) with CF

1 finding from 1 review

Psychological interventions for improving adherence to inhaled therapies in people with cystic fibrosis 2023 · Cochrane Headline outcomes only
no estimate extracted
1 study · 95 participants
Very low

Psychological interventions vs active comparators

FEV1 % predicted · adults (aged 16 years and over) with CF

1 finding from 1 review

Psychological interventions for improving adherence to inhaled therapies in people with cystic fibrosis 2023 · Cochrane Headline outcomes only
no estimate extracted
1 study · 125 participants
Very low

Psychological interventions vs active comparators

Pulmonary exacerbations · adults (aged 16 years and over) with CF

1 finding from 1 review

Psychological interventions for improving adherence to inhaled therapies in people with cystic fibrosis 2023 · Cochrane Headline outcomes only
no estimate extracted
1 study · 128 participants
Very low

Psychological interventions vs usual care

Treatment‐related adverse events Anxiety: GAD‐7 (Spitzer 2006) · children, adolescents or adults with CF

Summary of findings: psychological interventions showed no clear difference in treatment‐related adverse events Anxiety: GAD‐7 (Spitzer 2006) compared with usual care (low certainty).

1 finding from 1 review

Psychological interventions for improving adherence to inhaled therapies in people with cystic fibrosis 2023 · Cochrane Headline outcomes only
MD 0.3
1 study · 535 participants
Low