Findings

What do systematic reviews say?

632 questions · 636 findings · 33 reviews · Cystic Fibrosis and Genetic Disorders

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No studies found 197 · 31%GRADE not reported 13 · 2%Very low 133 · 20.9%Low 159 · 25%Moderate 121 · 19%High 13 · 2%

Summary of findings ‐ triple therapy: elexacaftor‐tezacaftor‐ivacaftor vs control (ivacaftor alone or tezacaftor plus ivacaftor)

Quality of life: CFQ‐R respiratory domain: absolute change from baseline · adults and children aged 12 years and over with cystic fibrosis and either F508del/gating variant or F508del/residual function genotype

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 ‐ triple therapy: elexacaftor‐tezacaftor‐ivacaftor vs control (ivacaftor alone or tezacaftor plus ivacaftor)

FEV1 % predicted: relative change from baseline · adults and children aged 12 years and over with cystic fibrosis and either F508del/gating variant or F508del/residual function genotype

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 ‐ triple therapy: elexacaftor‐tezacaftor‐ivacaftor vs control (ivacaftor alone or tezacaftor plus ivacaftor)

FEV1 % predicted: absolute change from baseline · adults and children aged 12 years and over with cystic fibrosis and either F508del/gating variant or F508del/residual function genotype

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 · 258 participants
Moderate

Summary of findings ‐ triple therapy: elexacaftor‐tezacaftor‐ivacaftor vs control (ivacaftor alone or tezacaftor plus ivacaftor)

Adverse events ‐ treatment emergent · adults and children aged 12 years and over with cystic fibrosis and either F508del/gating variant or F508del/residual function genotype

Summary of findings ‐ triple therapy: elexacaftor‐tezacaftor‐ivacaftor showed no clear difference in adverse events: number of participants with treatment‐emergent… compared with control (ivacaftor alone or tezacaftor plus ivacaftor) (moderate certainty).

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
OR 1.04 [0.53, 2.04]
1 study · 258 participants
Moderate

Summary of findings ‐ triple therapy: elexacaftor‐tezacaftor‐ivacaftor vs control (ivacaftor alone or tezacaftor plus ivacaftor)

Time to first pulmonary exacerbation · adults and children aged 12 years and over with cystic fibrosis and either F508del/gating variant or F508del/residual function genotype

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 ‐ 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

Summary of findings ‐ dual therapy: lumacaftor plus ivacaftor (twice daily) 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

Summary of findings ‐ dual therapy: lumacaftor plus ivacaftor (twice 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
1 study · 204 participants
Low

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

Summary of findings ‐ dual therapy: lumacaftor plus ivacaftor (twice 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
1 study · 204 participants
Low

Summary of findings ‐ dual therapy: lumacaftor plus ivacaftor (twice daily) 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 ‐ triple therapy: VX‐659‐tezacaftor‐ivacaftor/deutivacaftor vs control

Survival · adults with cystic fibrosis and either F508del/MF or F508del/F508del genotype

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
4 studies · 634 participants
Moderate