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Evidence found and GRADE certainty

134 questions · 134 findings · 11 reviews · Cystic Fibrosis and Genetic Disorders

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No studies found 31 · 23.1%GRADE not reported 10 · 7.5%Very low 23 · 17.2%Low 34 · 25.4%Moderate 36 · 26.9%High 0 · 0%

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

Adverse events ‐ All adverse events · people with inhibitors

Summary of findings table ‐ Concizumab prophylaxis showed no clear difference in adverse events ‐ All adverse events compared with on‐demand therapy (low certainty).

1 finding from 1 review

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

Adverse events ‐ Serious adverse events · people with inhibitors

Summary of findings table ‐ Concizumab prophylaxis showed no clear difference in adverse events ‐ Serious adverse events compared with on‐demand therapy (low 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 ‐ 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

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

All adverse events · people without inhibitors

Summary of findings table ‐ Emicizumab 1.5 mg/kg once weekly showed no clear difference in all adverse events compared with emicizumab 3.0 mg/kg every 2 weeks (moderate 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

Serious adverse events · people without inhibitors

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

1 finding from 1 review