Findings

What do systematic reviews say?

577 questions · 585 findings · 77 reviews · Cystic Fibrosis and Genetic Disorders

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No studies found 143 · 24.4%GRADE not reported 26 · 4.4%Very low 145 · 24.8%Low 156 · 26.7%Moderate 98 · 16.8%High 17 · 2.9%

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 All participants · 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 · 226 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

Inhaled corticosteroids vs placebo

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

1 finding from 1 review

Inhaled corticosteroids for cystic fibrosis 2019 · Cochrane Headline outcomes only
no estimate extracted
3 studies · 250 participants
Low

Inhaled corticosteroids vs placebo

FVC (% predicted) Change from baseline · adults and children with cystic fibrosis

1 finding from 1 review

Inhaled corticosteroids for cystic fibrosis 2019 · Cochrane Headline outcomes only
no estimate extracted
3 studies · 250 participants
Low

Intervention not reported

Lung function Mean percentage change in FVC (% predicted) · adults and children with cystic fibrosis

1 finding from 1 review

Once‐daily versus multiple‐daily dosing with intravenous aminoglycosides for cystic fibrosis 2019 · Cochrane Headline outcomes only
no estimate extracted
2 studies · 70 participants
Low

Summary of findings: oral antioxidants (NAC/GSH) vs placebo

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

2 findings from 1 review

Antioxidant supplementation for lung disease in cystic fibrosis 2019 · Cochrane Headline outcomes only
no estimate extracted
4 studies · 125 participants
Very low
Antioxidant supplementation for lung disease in cystic fibrosis 2019 · Cochrane Headline outcomes only
no estimate extracted
1 study · 62 participants
Moderate

Summary of findings: oral β‐carotene vs placebo

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

The review found no eligible studies for this question.

2 findings from 1 review

Antioxidant supplementation for lung disease in cystic fibrosis 2019 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found
Antioxidant supplementation for lung disease in cystic fibrosis 2019 · Cochrane Headline outcomes only
no estimate extracted
1 study · 24 participants
Very low

Summary of findings: oral antioxidant combination vs control

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

The review found no eligible studies for this question.

2 findings from 1 review

Antioxidant supplementation for lung disease in cystic fibrosis 2019 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found
Antioxidant supplementation for lung disease in cystic fibrosis 2019 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

Summary of findings: oral antioxidant combination vs control

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

The review found no eligible studies for this question.

2 findings from 1 review

Antioxidant supplementation for lung disease in cystic fibrosis 2019 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found
Antioxidant supplementation for lung disease in cystic fibrosis 2019 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

Summary of findings: inhaled antioxidants vs placebo

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

2 findings from 1 review

Antioxidant supplementation for lung disease in cystic fibrosis 2019 · Cochrane Headline outcomes only
no estimate extracted
3 studies · 258 participants
Moderate
Antioxidant supplementation for lung disease in cystic fibrosis 2019 · Cochrane Headline outcomes only
no estimate extracted
3 studies · 258 participants
Moderate

Summary of findings: oral vitamin E supplementation vs placebo or no treatment

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

The review found no eligible studies for this question.

2 findings from 1 review

Antioxidant supplementation for lung disease in cystic fibrosis 2019 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found
Antioxidant supplementation for lung disease in cystic fibrosis 2019 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

Intervention not reported

Mean percentage change from baseline in FVC (% predicted) · adults and children with mucopolysaccharidosis I

1 finding from 1 review

Enzyme replacement therapy with laronidase (Aldurazyme®) for treating mucopolysaccharidosis type I 2019 · Cochrane Headline outcomes only
no estimate extracted
1 study · 45 participants
Low

Summary of findings ‐ ivacaftor vs placebo

FEV1 % predicted: relative change from baseline · adults and children with cystic fibrosis and with at least one G551D CFTR mutation

1 finding from 1 review

Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis 2019 · Cochrane Headline outcomes only
no estimate extracted
3 studies · 232 participants
Moderate

Summary of findings ‐ ivacaftor vs placebo

FEV1 L and % predicted Absolute change from baseline · adults and children with cystic fibrosis and with at least one G551D CFTR mutation

1 finding from 1 review

Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis 2019 · Cochrane Headline outcomes only
no estimate extracted
3 studies · 232 participants
Low

Summary of findings ‐ ivacaftor vs placebo

FEV1 % predicted: relative change from baseline · adults and children with cystic fibrosis and with at least one R117H CFTR mutation

1 finding from 1 review

Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis 2019 · Cochrane Headline outcomes only
no estimate extracted
1 study · 69 participants
Moderate

Summary of findings ‐ ivacaftor vs placebo

FEV1 L and % predicted Absolute change from baseline · adults and children with cystic fibrosis and with at least one R117H CFTR mutation

1 finding from 1 review

Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis 2019 · Cochrane Headline outcomes only
no estimate extracted
1 study · 69 participants
Moderate

Summary of findings ‐ ivacaftor vs placebo

FEV1 % predicted: relative change from baseline · adults and children with cystic fibrosis and with the F508del CFTR mutation

1 finding from 1 review

Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis 2019 · Cochrane Headline outcomes only
no estimate extracted
1 study · 140 participants
Moderate

Summary of findings ‐ ivacaftor vs placebo

FEV1 % predicted: absolute change from baseline · adults and children with cystic fibrosis and with the F508del CFTR mutation

1 finding from 1 review

Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis 2019 · Cochrane Headline outcomes only
no estimate extracted
1 study · 140 participants
Moderate