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Evidence found and GRADE certainty

632 questions · 636 findings · 33 reviews · Cystic Fibrosis and Genetic Disorders

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No studies found 197 · 31%GRADE not reported 13 · 2%Very low 133 · 20.9%Low 159 · 25%Moderate 121 · 19%High 13 · 2%

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

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

Serious adverse events · people without inhibitors

Summary of findings table ‐ Emicuzimab 1.5 mg/kg weekly prophylaxis showed no clear difference in serious adverse events compared with on‐demand therapy (moderate 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 ‐ Emicuzimab 3.0 mg/kg bi‐weekly prophylaxis vs on‐demand therapy

All adverse events · people without inhibitors

Summary of findings table ‐ Emicuzimab 3.0 mg/kg bi‐weekly prophylaxis probably leads to higher all adverse events compared with on‐demand therapy (moderate certainty).

1 finding from 1 review

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

Serious adverse events · people without inhibitors

Summary of findings table ‐ Emicuzimab 3.0 mg/kg bi‐weekly prophylaxis showed no clear difference in serious adverse events 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 ‐ Fitusiran prophylaxis vs on‐demand therapy

Adverse events ‐ Serious adverse events · people without inhibitors

Summary of findings table ‐ Fitusiran prophylaxis showed no clear difference in adverse events ‐ Serious adverse events compared with on‐demand therapy (moderate certainty).

1 finding from 1 review