Findings

What do systematic reviews say?

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Evidence found and GRADE certainty

162 questions · 163 findings · 18 reviews · Cystic Fibrosis and Genetic Disorders

No studies found 47 · 28.8%GRADE not reported 0 · 0%Very low 28 · 17.2%Low 45 · 27.6%Moderate 35 · 21.5%High 8 · 4.9%

Summary of findings: aztreonam lysine for inhalation vs tobramycin

Need for antibiotics see pulmonary exacerbations · adults and children with cystic fibrosis and a positive microbiological isolate of P aeruginosa from a respiratory tract specimen

Summary of findings: aztreonam lysine for inhalation probably leads to lower pulmonary exacerbations: need for additional antibiotics compared with tobramycin (moderate 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

Cycled inhaled tobramycin vs culture‐based TNS

Adverse effects of antibiotics: proportion of participants with one or more serious adverse event · adults and children with cystic fibrosis and a positive microbiological isolate of P aeruginosa from a respiratory tract specimen

Cycled inhaled tobramycin (TNS) probably leads to lower adverse effects of antibiotics: proportion of participants with one… compared with culture‐based TNS (moderate certainty).

1 finding from 1 review

Ciprofloxacin vs placebo added to cycled and culture‐based inhaled tobramycin (TNS) therapy

Adverse effects of antibiotics: proportion of participants with one or more serious adverse event · adults and children with cystic fibrosis and a positive microbiological isolate of P aeruginosa from a respiratory tract specimen

Ciprofloxacin showed no clear difference in adverse effects of antibiotics: proportion of participants with one… compared with placebo added to cycled and culture‐based inhaled tobramycin (TNS)… (moderate certainty).

1 finding from 1 review

Oral ciprofloxacin and inhaled colistin vs inhaled tobramycin

Adverse effects of antibiotics: severe cough · adults and children with cystic fibrosis and a positive microbiological isolate of P aeruginosa from a respiratory tract specimen

It is very uncertain whether oral ciprofloxacin and inhaled colistin changes adverse effects of antibiotics: severe cough compared with inhaled tobramycin (TNS) (very low certainty).

1 finding from 1 review

Inhaled tobramycin (TNS) plus oral azithromycin vs TNS plus oral placebo

Number experiencing adverse effects · children with cystic fibrosis and a new culture positive for P aeruginosa

Inhaled tobramycin (TNS) plus oral azithromycin showed no clear difference in adverse effects of antibiotics: number of participants experiencing… compared with TNS plus oral placebo (moderate certainty).

1 finding from 1 review

Inhaled tobramycin (TNS) vs placebo

Adverse effects of antibiotics: participants experiencing cough · adults and children with cystic fibrosis and a positive microbiological isolate of P aeruginosa from a respiratory tract specimen

It is very uncertain whether inhaled tobramycin (TNS) changes adverse effects of antibiotics: participants experiencing cough compared with placebo (very low certainty).

1 finding from 1 review

Intravenous ceftazidime with tobramycin vs ciprofloxacin

Adverse effects of antibiotics · adults and children with cystic fibrosis and a positive microbiological isolate of P aeruginosa from a respiratory tract specimen

Intravenous ceftazidime with tobramycin probably leads to lower adverse effects of antibiotics: compared with oral ciprofloxacin (moderate certainty).

1 finding from 1 review

Short‐acting inhaled beta‐2 agonists vs placebo

Number of exacerbations(as reported by number of hospitalisations and number of courses and days on antibiotics) · adults and children with cystic fibrosis

It is very uncertain whether short‐acting inhaled beta‐2 agonists changes number of exacerbations(as reported by number of hospitalisations… compared with placebo (very low certainty).

1 finding from 1 review

Summary of findings: Single vs combination intravenous anti‐pseudomonal antibiotic therapy

Time to next course of antibiotics  · adults and children with cystic fibrosis

The review found no eligible studies for this question.

2 findings from 1 review