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Evidence found and GRADE certainty

577 questions · 585 findings · 77 reviews · Cystic Fibrosis and Genetic Disorders

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No studies found 143 · 24.4%GRADE not reported 26 · 4.4%Very low 145 · 24.8%Low 156 · 26.7%Moderate 98 · 16.8%High 17 · 2.9%

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

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