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Evidence found and GRADE certainty

7 questions · 7 findings · 1 reviews · Cystic Fibrosis and Genetic Disorders

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No studies found 0 · 0%GRADE not reported 0 · 0%Very low 0 · 0%Low 3 · 42.9%Moderate 4 · 57.1%High 0 · 0%

Summary of findings table ‐ Fitusiran prophylaxis vs on‐demand therapy

Proportion with zero bleeds · people with inhibitors

Summary of findings table ‐ Fitusiran prophylaxis probably leads to higher proportion with zero bleeds compared with on‐demand therapy (moderate certainty).

1 finding from 1 review

Summary of findings table ‐ Emicizumab prophylaxis vs on‐demand therapy

Proportion with zero bleeds · people with inhibitors

Summary of findings table ‐ Emicizumab prophylaxis probably leads to higher proportion with zero bleeds compared with on‐demand therapy (moderate certainty).

1 finding from 1 review

Summary of findings table ‐ Fitusiran prophylaxis vs on‐demand therapy

Proportion with zero bleeds · people without inhibitors

Summary of findings table ‐ Fitusiran prophylaxis probably leads to higher proportion with zero bleeds compared with on‐demand therapy (moderate certainty).

1 finding from 1 review

Summary of findings table ‐ Emicuzimab 1.5 mg/kg weekly prophylaxis vs on‐demand therapy

Proportion with zero bleeds · people without inhibitors

Summary of findings table ‐ Emicuzimab 1.5 mg/kg weekly prophylaxis may leads to higher proportion of participants with zero bleeds compared with on‐demand therapy (low certainty).

1 finding from 1 review

Summary of findings table ‐ Emicuzimab 3.0 mg/kg bi‐weekly prophylaxis vs on‐demand therapy

Proportion with zero bleeds · people without inhibitors

Summary of findings table ‐ Emicuzimab 3.0 mg/kg bi‐weekly prophylaxis showed no clear difference in proportion of participants with zero bleeds compared with on‐demand therapy (low certainty).

1 finding from 1 review

Summary of findings table ‐ Emicizumab 1.5 mg/kg once weekly vs emicizumab 3.0 mg/kg every 2 weeks

Proportion with zero bleeds · people without inhibitors

Summary of findings table ‐ Emicizumab 1.5 mg/kg once weekly showed no clear difference in proportion of participants with zero bleeds compared with emicizumab 3.0 mg/kg every 2 weeks (moderate certainty).

1 finding from 1 review