Findings

What do systematic reviews say?

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Evidence found and GRADE certainty

697 questions · 708 findings · 47 reviews · Cystic Fibrosis and Genetic Disorders

No studies found 263 · 37.1%GRADE not reported 12 · 1.7%Very low 146 · 20.6%Low 168 · 23.7%Moderate 98 · 13.8%High 21 · 3%

Inhaled NO vs placebo

Frequency of pain crises in the · people with SCD

Inhaled nitric oxide showed no clear difference in frequency of pain crises in the compared with placebo (low certainty).

2 findings from 1 review

Short‐acting inhaled beta‐2 agonists vs placebo

Number of exacerbations(as reported by number of hospitalisations and number of courses and days on antibiotics) · adults and children with cystic fibrosis

It is very uncertain whether short‐acting inhaled beta‐2 agonists changes number of exacerbations(as reported by number of hospitalisations… compared with placebo (very low certainty).

1 finding from 1 review

Short‐acting inhaled beta‐2 agonists vs placebo

Participant‐reported symptom score: cough, wheeze and exercise tolerance · adults and children with cystic fibrosis

It is very uncertain whether short‐acting inhaled beta‐2 agonists changes participant‐reported symptom score: cough, wheeze and exercise… compared with placebo (very low certainty).

1 finding from 1 review