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Evidence found and GRADE certainty

4,860 questions · 64,864 findings · 4,913 reviews · Headline outcomes only

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No studies found 13,869 · 21.4%GRADE not reported 3,491 · 5.4%Very low 16,300 · 25.1%Low 17,338 · 26.7%Moderate 10,900 · 16.8%High 2,966 · 4.6%

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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 ‐ 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 ‐ 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 1.5 mg/kg weekly prophylaxis vs on‐demand therapy

All adverse events · people without inhibitors

Summary of findings table ‐ Emicuzimab 1.5 mg/kg weekly prophylaxis probably leads to higher all adverse events compared with on‐demand therapy (moderate certainty).

1 finding from 1 review