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Evidence found and GRADE certainty

2,261 questions · 2,280 findings · 114 reviews · Cystic Fibrosis and Genetic Disorders

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No studies found 856 · 37.5%GRADE not reported 58 · 2.5%Very low 550 · 24.1%Low 502 · 22%Moderate 271 · 11.9%High 43 · 1.9%

Dietary advice vs oral nutritional supplements

Number of people admitted or re‐admitted to hospital · adults with disease‐related malnutrition

Dietary advice showed no clear difference in number of people admitted or re‐admitted to hospital compared with oral nutritional supplements (low 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 ‐ withdrawal of penicillin prophylaxis vs continuation

Requirement of other courses of antibiotics · 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 requirement of other courses of antibiotics compared with continuation (low certainty).

1 finding from 1 review

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

Recombinant human growth hormone vs placebo or no intervention

Measures of mineral metabolism: change in serum phosphate mmol/L · children with X‐linked hypophosphataemia

It is very uncertain whether summary of findings ‐ recombinant growth hormone changes measures of mineral metabolism: change in serum phosphate mmol/L compared with placebo or no intervention (very low certainty).

1 finding from 1 review