Findings

What do systematic reviews say?

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Evidence found and GRADE certainty

134 questions · 134 findings · 11 reviews · Cystic Fibrosis and Genetic Disorders

No studies found 31 · 23.1%GRADE not reported 10 · 7.5%Very low 23 · 17.2%Low 34 · 25.4%Moderate 36 · 26.9%High 0 · 0%

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 ‐ Prophylactic vs 'as required' anti‐staphylococcal antibiotics

Number of people with one or more isolates of S aureus (sensitive strains) · children with cystic fibrosis

Summary of findings ‐ Prophylactic may leads to lower number of people with one or more isolates of S aureus (sensitive… compared with 'as required' anti‐staphylococcal antibiotics (low certainty).

1 finding from 1 review

Summary of findings ‐ Prophylactic vs 'as required' anti‐staphylococcal antibiotics

Number of people with one or more isolates of P aeruginosa · children with cystic fibrosis

Summary of findings ‐ Prophylactic showed no clear difference in number of people with one or more isolates of P aeruginosa compared with 'as required' anti‐staphylococcal antibiotics (low certainty).

1 finding from 1 review

Summary of findings ‐ Prophylactic vs 'as required' anti‐staphylococcal antibiotics

Need for additional antibiotic therapy · children with cystic fibrosis

Summary of findings ‐ Prophylactic showed no clear difference in number of people needing additional antibiotics compared with 'as required' anti‐staphylococcal antibiotics (low certainty).

1 finding from 1 review

Summary of findings: cycled vs culture‐based antibiotic therapy

Emergence of novel bacteria: isolation of novel gram negative organisms · children (aged 1 to 12 years) with CF and a newly isolated PA infection

Summary of findings: cycled showed no clear difference in emergence of novel bacteria: isolation of novel gram negative… compared with culture‐based antibiotic therapy (moderate certainty).

1 finding from 1 review

Summary of findings ‐ initiation of penicillin prophylaxis vs placebo

Incidence of S pneumoniae infection Isolated bacterial infection · children with SCD

Summary of findings ‐ initiation of penicillin prophylaxis may leads to lower incidence of S pneumoniae infection Isolated bacterial infection compared with placebo (low certainty).

1 finding from 1 review

Summary of findings ‐ DVD plus information booklet vs information booklet alone

Compliance or adherence to the trial intervention (including satisfaction): use of intervention at least once · adults and children with hemophilia

Summary of findings ‐ DVD plus information booklet showed no clear difference in compliance or adherence to the trial intervention (including… compared with information booklet alone (low certainty).

1 finding from 1 review