Findings

What do systematic reviews say?

2,261 questions · 2,280 findings · 114 reviews · Cystic Fibrosis and Genetic Disorders

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No studies found 856 · 37.5%GRADE not reported 58 · 2.5%Very low 550 · 24.1%Low 502 · 22%Moderate 271 · 11.9%High 43 · 1.9%

Summary of findings: oral antioxidant combination vs control

Time to next exacerbation · adults and children with cystic fibrosis

1 finding from 1 review

Antioxidant supplementation for lung disease in cystic fibrosis 2019 · Cochrane Headline outcomes only
no estimate extracted
1 study · 69 participants
Low

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

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

1 finding from 1 review

Antioxidant supplementation for lung disease in cystic fibrosis 2019 · Cochrane Headline outcomes only
no estimate extracted
1 study · 61 participants
Moderate

Summary of findings: oral antioxidant combination vs control

Adverse events · adults and children with cystic fibrosis

1 finding from 1 review

Antioxidant supplementation for lung disease in cystic fibrosis 2019 · Cochrane Headline outcomes only
no estimate extracted
1 study · 69 participants
Low

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: inhaled antioxidants vs placebo

Quality of life: change in CFQoL score from baseline · adults and children with cystic fibrosis

1 finding from 1 review

Antioxidant supplementation for lung disease in cystic fibrosis 2019 · Cochrane Headline outcomes only
no estimate extracted
1 study · 153 participants
Moderate

Summary of findings: inhaled antioxidants vs placebo

Change in BMI · adults and children with cystic fibrosis

The review found no eligible studies for this question.

1 finding 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

Summary of findings: inhaled antioxidants vs placebo

Time to next exacerbation · adults and children with cystic fibrosis

The review found no eligible studies for this question.

1 finding 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

Summary of findings: inhaled antioxidants vs placebo

Adverse events · adults and children with cystic fibrosis

1 finding from 1 review

Antioxidant supplementation for lung disease in cystic fibrosis 2019 · Cochrane Headline outcomes only
no estimate extracted
3 studies · 223 participants
Low

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

Change in BMI · adults and children with cystic fibrosis

1 finding from 1 review

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 antioxidants (NAC/GSH) vs placebo

Time to next exacerbation · adults and children with cystic fibrosis

The review found no eligible studies for this question.

1 finding 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

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

Adverse events · adults and children with cystic fibrosis

Summary of findings: oral antioxidants (NAC/GSH) showed no clear difference in adverse events compared with placebo (moderate certainty).

1 finding from 1 review

Antioxidant supplementation for lung disease in cystic fibrosis 2019 · Cochrane Headline outcomes only
OR 2.92 [0.11, 74.05]
1 study · 70 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

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

Quality of life · adults and children with cystic fibrosis

The review found no eligible studies for this question.

1 finding 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

Intervention not reported

Change in urinary GAG excretion from baseline · 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 · 43 participants
Low

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

Intervention not reported

Mean change from baseline in 6MWT (metres) · 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

Intervention not reported

Infusion‐related reactions (number of participants with one or more reaction) · adults and children with mucopolysaccharidosis I

The intervention showed no clear difference in infusion‐related reactions (number of participants with one or more… (low certainty).

1 finding from 1 review

Enzyme replacement therapy with laronidase (Aldurazyme®) for treating mucopolysaccharidosis type I 2019 · Cochrane Headline outcomes only
OR 0.51 [0.15, 1.71]
1 study · 45 participants
Low

Intervention not reported

Antibody production: number of participants developing IgG antibodies · adults and children with mucopolysaccharidosis I

It is very uncertain whether the intervention changes antibody production: number of participants developing IgG antibodies (very low certainty).

1 finding from 1 review

Enzyme replacement therapy with laronidase (Aldurazyme®) for treating mucopolysaccharidosis type I 2019 · Cochrane Headline outcomes only
OR 385.4 [17.47, 8500.51]
1 study · 45 participants
Very low

Intervention not reported

Quality of life · 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

Intervention not reported

Mortality: number of deaths · 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