Findings

What do systematic reviews say?

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Evidence found and GRADE certainty

873 questions · 883 findings · 38 reviews · Cystic Fibrosis and Genetic Disorders

No studies found 319 · 36.1%GRADE not reported 18 · 2%Very low 198 · 22.4%Low 186 · 21.1%Moderate 147 · 16.6%High 15 · 1.7%

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

NSAIDs vs placebo

Transmission‐related outcomes: hospitalization ‐ Number or proportion of hospital admissions · adults and children with cystic fibrosis

Summary of findings: non‐steroidal anti‐inflammatory drugs showed no clear difference in proportion with at least 1 hospital admission compared with placebo (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