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%

Intervention not reported

Infusion‐related events · infantile‐onset Pompe disease

The intervention showed no clear difference in infusion‐related events (low certainty).

1 finding from 1 review

Enzyme replacement therapy for infantile‐onset Pompe disease 2017 · Cochrane Headline outcomes only
RR 0.83 [0.4, 1.76]
1 study · 18 participants
Low

Intervention not reported

Other adverse events · infantile‐onset Pompe disease

1 finding from 1 review

Enzyme replacement therapy for infantile‐onset Pompe disease 2017 · Cochrane Headline outcomes only
no estimate extracted
1 study · 18 participants
Low

Beta‐2 agonists vs placebo

Lung function: FEV1 mL (change from baseline) · adults and children with cystic fibrosis

1 finding from 1 review

Long‐acting inhaled bronchodilators for cystic fibrosis 2017 · Cochrane Headline outcomes only
no estimate extracted
1 study · 16 participants
Very low

LAMA vs placebo

Other measures of lung function: mean change from baseline in FEV1 AUC0-4h (% predicted) after tiotropium 5.0 µg · adults and children with cystic fibrosis

1 finding from 1 review

Long‐acting inhaled bronchodilators for cystic fibrosis 2017 · Cochrane Headline outcomes only
no estimate extracted
2 studies · 770 participants
Moderate

LAMA vs placebo

Change in baseline trough FEV1 · adults and children with cystic fibrosis

1 finding from 1 review

Long‐acting inhaled bronchodilators for cystic fibrosis 2017 · Cochrane Headline outcomes only
no estimate extracted
2 studies · 772 participants
Moderate

Beta‐2 agonists vs placebo

Participant‐reported outcomes: quality of life (measured by CFQ‐R) · adults and children with cystic fibrosis

The review found no eligible studies for this question.

1 finding from 1 review

Long‐acting inhaled bronchodilators for cystic fibrosis 2017 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

Beta‐2 agonists vs placebo

Adverse events after tiotropium 5.0 µg · adults and children with cystic fibrosis

The review found no eligible studies for this question.

1 finding from 1 review

Long‐acting inhaled bronchodilators for cystic fibrosis 2017 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

Beta‐2 agonists vs placebo

Pulmonary exacerbations · adults and children with cystic fibrosis

The review found no eligible studies for this question.

1 finding from 1 review

Long‐acting inhaled bronchodilators for cystic fibrosis 2017 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

Beta‐2 agonists vs placebo

Other measures of lung function · adults and children with cystic fibrosis

The review found no eligible studies for this question.

1 finding from 1 review

Long‐acting inhaled bronchodilators for cystic fibrosis 2017 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

LAMA vs placebo

Lung function: FEV1 mL (change from baseline) · adults and children with cystic fibrosis

1 finding from 1 review

Long‐acting inhaled bronchodilators for cystic fibrosis 2017 · Cochrane Headline outcomes only
no estimate extracted
1 study · 34 participants
Low

LAMA vs placebo

Participant‐reported outcomes: quality of life (measured by CFQ‐R) · adults and children with cystic fibrosis

1 finding from 1 review

Long‐acting inhaled bronchodilators for cystic fibrosis 2017 · Cochrane Headline outcomes only
no estimate extracted
2 studies · 974 participants
Low

LAMA vs placebo

Adverse events after tiotropium 5.0 µg · adults and children with cystic fibrosis

LAMAs showed no clear difference in adverse events after tiotropium 5.0 µg compared with placebo (high certainty).

1 finding from 1 review

Long‐acting inhaled bronchodilators for cystic fibrosis 2017 · Cochrane Headline outcomes only
RR 0.96 [0.88, 1.04]
2 studies · 807 participants
High

LAMA vs placebo

Pulmonary exacerbations · adults and children with cystic fibrosis

LAMAs showed no clear difference in pulmonary exacerbations: participants reporting ≥1 pulmonary… compared with placebo (moderate certainty).

1 finding from 1 review

Long‐acting inhaled bronchodilators for cystic fibrosis 2017 · Cochrane Headline outcomes only
RR 0.6 [0.32, 1.12]
2 studies · 805 participants
Moderate

Intervention not reported

Changes in globotriaosylceramide (Gb3) concentration in plasma · Anderson‐Fabry disease

1 finding from 1 review

Enzyme replacement therapy for Anderson‐Fabry disease 2016 · Cochrane Headline outcomes only
no estimate extracted
2 studies · 39 participants
Very low

Intervention not reported

Renal function events (ITT population) · Anderson‐Fabry disease

1 finding from 1 review

Enzyme replacement therapy for Anderson‐Fabry disease 2016 · Cochrane Headline outcomes only
no estimate extracted
1 study · 82 participants
Very low

Intervention not reported

Symptoms and complications of disease: cardiac events · Anderson‐Fabry disease

1 finding from 1 review

Enzyme replacement therapy for Anderson‐Fabry disease 2016 · Cochrane Headline outcomes only
no estimate extracted
1 study · 82 participants
Very low

Intervention not reported

Symptoms and complications of disease: cerebrovascular events (ITT population) · Anderson‐Fabry disease

1 finding from 1 review

Enzyme replacement therapy for Anderson‐Fabry disease 2016 · Cochrane Headline outcomes only
no estimate extracted
1 study · 82 participants
Very low

Intervention not reported

Changes in globotriaosylceramide (Gb3) concentration in plasma and tissue · Anderson‐Fabry disease

The review found no eligible studies for this question.

1 finding from 1 review

Enzyme replacement therapy for Anderson‐Fabry disease 2016 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

Intervention not reported

Death · Anderson‐Fabry disease

1 finding from 1 review

Enzyme replacement therapy for Anderson‐Fabry disease 2016 · Cochrane Headline outcomes only
no estimate extracted
1 study · 36 participants
Very low

Intervention not reported

Pain Acroparaesthesia and Fabry crises · Anderson‐Fabry disease

The review found no eligible studies for this question.

1 finding from 1 review

Enzyme replacement therapy for Anderson‐Fabry disease 2016 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found