Findings

What do systematic reviews say?

42 questions · 42 findings · 7 reviews · Cystic Fibrosis and Genetic Disorders · Headline outcomes only

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No studies found 13 · 31%GRADE not reported 0 · 0%Very low 5 · 11.9%Low 9 · 21.4%Moderate 15 · 35.7%High 0 · 0%

Digital technology (web‐based platform CFHealthHub) vs usual care

Adherence to the inhaled treatment: percentage adherence · adults (aged 16 years and over) with CF

Digital technology (web‐based platform CFHealthHub) probably leads to higher adherence to the inhaled treatment: percentage adherence compared with usual care (moderate certainty).

1 finding from 1 review

Digital technology for monitoring adherence to inhaled therapies in people with cystic fibrosis 2023 · Cochrane Headline outcomes only
MD 18 [12.9, 23.1]
1 study · 588 participants
Moderate

NSAIDs vs placebo

Annual rate of change in % predicted FEV1 Subgroup: under 13 years of age · adults and children with cystic fibrosis

1 finding from 1 review

Oral non‐steroidal anti‐inflammatory drug therapy for lung disease in cystic fibrosis 2019 · Cochrane Headline outcomes only
no estimate extracted
2 studies · 147 participants
Moderate

NSAIDs vs placebo

Annual rate of change in % predicted FEV1 Subgroup: over 13 years of age · adults and children with cystic fibrosis

1 finding from 1 review

Oral non‐steroidal anti‐inflammatory drug therapy for lung disease in cystic fibrosis 2019 · Cochrane Headline outcomes only
no estimate extracted
2 studies · 79 participants
Moderate

NSAIDs vs placebo

Annual rate of change in % predicted FVC Subgroup: Under 13 years of age · adults and children with cystic fibrosis

1 finding from 1 review

Oral non‐steroidal anti‐inflammatory drug therapy for lung disease in cystic fibrosis 2019 · Cochrane Headline outcomes only
no estimate extracted
2 studies · 147 participants
Moderate

NSAIDs vs placebo

Annual rate of change in % predicted FVC Subgroup: Over 13 years of age · adults and children with cystic fibrosis

1 finding from 1 review

Oral non‐steroidal anti‐inflammatory drug therapy for lung disease in cystic fibrosis 2019 · Cochrane Headline outcomes only
no estimate extracted
2 studies · 79 participants
Moderate

Intervention not reported

Change in measures of growth and maturation: change in puberty proportion with Tanner stage ≧ 1 level ‐ at 2 years · children with familial hypercholesterolemia

The intervention showed no clear difference in change in measures of growth and maturation: change in puberty… (low certainty).

1 finding from 1 review

Statins for children with familial hypercholesterolemia 2019 · Cochrane Headline outcomes only
RR 0.95 [0.77, 1.18]
1 study · 211 participants
Low

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

Survival · 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)

Quality of life: total score · 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)

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

Digital technology (TIM) vs standard treatment (TBM)

Adherence to inhaled therapies cMPR · children aged 5 to 16 years

Digital technology (TIM) may leads to higher adherence to the inhaled treatment: compared with standard treatment (TBM) (low certainty).

1 finding from 1 review

Digital technology for monitoring adherence to inhaled therapies in people with cystic fibrosis 2023 · Cochrane Headline outcomes only
MD 24 [2.95, 45.05]
1 study · 20 participants
Low

Digital technology (web‐based platform CFHealthHub) vs usual care

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

Digital technology (web‐based platform CFHealthHub) probably leads to lower number of pulmonary exacerbations compared with usual care (moderate certainty).

1 finding from 1 review

Digital technology for monitoring adherence to inhaled therapies in people with cystic fibrosis 2023 · Cochrane Headline outcomes only
rate ratio 0.92
1 study · 607 participants
Moderate

Digital technology (TIM) vs standard treatment (TBM)

Treatment burden · children aged 5 to 16 years

The review found no eligible studies for this question.

1 finding from 1 review

Digital technology for monitoring adherence to inhaled therapies in people with cystic fibrosis 2023 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

Digital technology (TIM) vs standard treatment (TBM)

QoL · children aged 5 to 16 years

The review found no eligible studies for this question.

1 finding from 1 review

Digital technology for monitoring adherence to inhaled therapies in people with cystic fibrosis 2023 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

Digital technology (TIM) vs standard treatment (TBM)

FEV1 (% predicted): mean change from baseline · children aged 5 to 16 years

Digital technology (TIM) showed no clear difference in FEV1 (mean change from baseline, % predicted) compared with standard treatment (TBM) (low certainty).

1 finding from 1 review

Digital technology for monitoring adherence to inhaled therapies in people with cystic fibrosis 2023 · Cochrane Headline outcomes only
MD 1
1 study · 20 participants
Low

Digital technology (TIM) vs standard treatment (TBM)

Pulmonary exacerbations · children aged 5 to 16 years

The review found no eligible studies for this question.

1 finding from 1 review

Digital technology for monitoring adherence to inhaled therapies in people with cystic fibrosis 2023 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

Digital technology (web‐based platform CFHealthHub) vs usual care

Treatment burden · adults (aged 16 years and over) with CF

Digital technology (web‐based platform CFHealthHub) probably leads to higher treatment burden: CFQ‐R treatment burden domain score (higher score… compared with usual care (moderate certainty).

1 finding from 1 review

Digital technology for monitoring adherence to inhaled therapies in people with cystic fibrosis 2023 · Cochrane Headline outcomes only
MD 5.1 [1.79, 8.41]
1 study · 539 participants
Moderate