Findings

What do systematic reviews say?

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Evidence found and GRADE certainty

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

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 ‐ withdrawal of penicillin prophylaxis vs continuation

Incidence of S pneumoniae Confirmed bacterial infection · children with SCD who have been receiving prophylactic penicillin for at least two years

Summary of findings ‐ withdrawal of penicillin prophylaxis showed no clear difference in incidence of S pneumoniae Confirmed bacterial infection compared with continuation (low certainty).

1 finding from 1 review

Summary of findings ‐ withdrawal of penicillin prophylaxis vs continuation

Nausea and vomiting · children with SCD who have been receiving prophylactic penicillin for at least two years

Summary of findings ‐ withdrawal of penicillin prophylaxis showed no clear difference in adverse effects: incidences of nausea and vomiting compared with continuation (low certainty).

1 finding from 1 review

Prophylaxis with standard therapeutic factor concentrate vs pegylated liposome FVIII formulation

Annualized joint Bleeding Rate (AjBR) · children or adults with hemophilia A

Prophylaxis with standard therapeutic factor concentrate showed no clear difference in AJBR compared with pegylated liposome FVIII formulation (low certainty).

1 finding from 1 review

Prophylaxis with standard therapeutic factor concentrate vs pegylated liposome FVIII formulation

ABR · children or adults with hemophilia A

Prophylaxis with standard therapeutic factor concentrate showed no clear difference in ABR compared with pegylated liposome FVIII formulation (low certainty).

1 finding from 1 review