Findings

What do systematic reviews say?

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Evidence found and GRADE certainty

11 questions · 11 findings · 2 reviews · Cystic Fibrosis and Genetic Disorders

No studies found 7 · 63.6%GRADE not reported 0 · 0%Very low 0 · 0%Low 0 · 0%Moderate 4 · 36.4%High 0 · 0%

Summary of findings ‐ ivacaftor vs placebo

Time to first pulmonary exacerbation · adults and children with cystic fibrosis and with at least one G551D CFTR mutation

Summary of findings ‐ ivacaftor probably leads to lower time to first pulmonary exacerbation compared with placebo (moderate certainty).

1 finding from 1 review

Summary of findings ‐ ivacaftor vs placebo

Time to first pulmonary exacerbation · adults and children with cystic fibrosis and with at least one R117H CFTR mutation

Summary of findings ‐ ivacaftor showed no clear difference in time to first pulmonary exacerbation compared with placebo (moderate certainty).

1 finding from 1 review