Findings

What do systematic reviews say?

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

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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 for combination antimicrobial susceptibility testing vs conventional treatment (separate testing)

Lung function(FEV1 or FVC L/min or % predicted) · adults and children with pulmonary exacerbation due to Pseudomonas aeruginosa

The review found no eligible studies for this question.

1 finding from 1 review

Combination antimicrobial susceptibility testing for acute exacerbations in chronic infection of Pseudomonas… 2020 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

Summary of findings for combination antimicrobial susceptibility testing vs conventional treatment (separate testing)

Time to next exacerbation · adults and children with pulmonary exacerbation due to Pseudomonas aeruginosa

1 finding from 1 review

Combination antimicrobial susceptibility testing for acute exacerbations in chronic infection of Pseudomonas… 2020 · Cochrane Headline outcomes only
no estimate extracted
1 study · 82 participants
Moderate

Summary of findings for combination antimicrobial susceptibility testing vs conventional treatment (separate testing)

Quality of life · adults and children with pulmonary exacerbation due to Pseudomonas aeruginosa

The review found no eligible studies for this question.

1 finding from 1 review

Combination antimicrobial susceptibility testing for acute exacerbations in chronic infection of Pseudomonas… 2020 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

Summary of findings for combination antimicrobial susceptibility testing vs conventional treatment (separate testing)

Length of hospital stay · adults and children with pulmonary exacerbation due to Pseudomonas aeruginosa

The review found no eligible studies for this question.

1 finding from 1 review

Combination antimicrobial susceptibility testing for acute exacerbations in chronic infection of Pseudomonas… 2020 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

Summary of findings for combination antimicrobial susceptibility testing vs conventional treatment (separate testing)

Sputum bacterial density (CFU/mL) · adults and children with pulmonary exacerbation due to Pseudomonas aeruginosa

The review found no eligible studies for this question.

1 finding from 1 review

Combination antimicrobial susceptibility testing for acute exacerbations in chronic infection of Pseudomonas… 2020 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

Summary of findings for combination antimicrobial susceptibility testing vs conventional treatment (separate testing)

Adverse events · adults and children with pulmonary exacerbation due to Pseudomonas aeruginosa

The review found no eligible studies for this question.

1 finding from 1 review

Combination antimicrobial susceptibility testing for acute exacerbations in chronic infection of Pseudomonas… 2020 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

Summary of findings for combination antimicrobial susceptibility testing vs conventional treatment (separate testing)

All‐cause mortality · adults and children with pulmonary exacerbation due to Pseudomonas aeruginosa

The review found no eligible studies for this question.

1 finding from 1 review

Combination antimicrobial susceptibility testing for acute exacerbations in chronic infection of Pseudomonas… 2020 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

DFX film‐coated tablet vs DFX dispersible tablet

Adherence to iron chelation therapy (%, SD)

1 finding from 1 review

Interventions for improving adherence to iron chelation therapy in people with sickle cell disease or thalassaemia 2023 · Cochrane Headline outcomes only
no estimate extracted
1 study · 91 participants
Very low

DFX film‐coated tablet vs DFX dispersible tablet

Sustained adherence to iron chelation therapy (%, SD)

1 finding from 1 review

Interventions for improving adherence to iron chelation therapy in people with sickle cell disease or thalassaemia 2023 · Cochrane Headline outcomes only
no estimate extracted
1 study · 54 participants
Very low

DFX film‐coated tablet vs DFX dispersible tablet

SAEs

It is very uncertain whether summary of findings: Comparison 4 ‐ deferasirox (DFX) film‐coated… changes incidence of SAEs compared with DFX dispersible tablets (very low certainty).

1 finding from 1 review

Interventions for improving adherence to iron chelation therapy in people with sickle cell disease or thalassaemia 2023 · Cochrane Headline outcomes only
RR 1.22 [0.62, 2.37]
1 study · 173 participants
Very low

DFX film‐coated tablet vs DFX dispersible tablet

All‐cause mortality

1 finding from 1 review

Interventions for improving adherence to iron chelation therapy in people with sickle cell disease or thalassaemia 2023 · Cochrane Headline outcomes only
7.3 [0.14, 368.15]
1 study · 173 participants
Very low

DFX film‐coated tablet vs DFX dispersible tablet

QoL

The review found no eligible studies for this question.

1 finding from 1 review

Interventions for improving adherence to iron chelation therapy in people with sickle cell disease or thalassaemia 2023 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

Summary of findings ‐ dual therapy: lumacaftor plus ivacaftor (once daily) 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
2 studies · 1108 participants
High

Summary of findings ‐ dual therapy: lumacaftor plus ivacaftor (once daily) vs placebo

Quality of life ‐ (EuroQol) EQ‐5D‐3L Index Score (total score): 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
2 studies · 1061 participants
Moderate

Summary of findings ‐ dual therapy: lumacaftor plus ivacaftor (once daily) 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
3 studies · 1139 participants
Moderate

Summary of findings ‐ dual therapy: lumacaftor plus ivacaftor (once daily) vs placebo

FEV1 % predicted: relative 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
3 studies · 1134 participants
High

Summary of findings ‐ dual therapy: lumacaftor plus ivacaftor (once daily) 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
3 studies · 1134 participants
Moderate

Summary of findings ‐ dual therapy: lumacaftor plus ivacaftor (once daily) 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 · 1108 participants
High

Summary of findings ‐ dual therapy: lumacaftor plus ivacaftor (once daily) vs placebo

Time to first pulmonary exacerbation · 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 · 1108 participants
Moderate

Summary of findings ‐ dual therapy: lumacaftor plus ivacaftor (twice daily) 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 · 204 participants
Moderate