Findings

What do systematic reviews say?

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Evidence found and GRADE certainty

697 questions · 708 findings · 47 reviews · Cystic Fibrosis and Genetic Disorders

No studies found 263 · 37.1%GRADE not reported 12 · 1.7%Very low 146 · 20.6%Low 168 · 23.7%Moderate 98 · 13.8%High 21 · 3%

Single foetal haemoglobin inducer at any dose or duration vs placebo or usual care

Adverse events · people with non‐transfusion‐dependent β‐thalassaemia

It is very uncertain whether single foetal haemoglobin inducer at any dose or duration changes adverse events compared with usual care or placebo (very low certainty).

1 finding from 1 review

Summary of findings: anti‐pseudomonal antibiotics vs placebo

Frequency of hospital admissions for pulmonary exacerbations ‐ not measured · adults and children with cystic fibrosis and a positive microbiological isolate of P aeruginosa from a respiratory tract specimen

Summary of findings: anti‐pseudomonal antibiotics may leads to lower pulmonary exacerbations: frequency of one or more hospital admissions compared with placebo (low certainty).

1 finding from 1 review

Summary of findings: anti‐pseudomonal antibiotics vs placebo

Survival: number of deaths · adults and children with cystic fibrosis and a positive microbiological isolate of P aeruginosa from a respiratory tract specimen

Summary of findings: anti‐pseudomonal antibiotics showed no clear difference in survival: number of deaths compared with placebo (low certainty).

1 finding from 1 review

Summary of findings: continuous cycles alternating aztreonam lysine for inhalation with tobramycin for inhalation solution vs continuous cycles alternating placebo with tobramycin

FEV1 (% predicted): mean change from baseline · adults and children with cystic fibrosis and a positive microbiological isolate of P aeruginosa from a respiratory tract specimen
1 finding from 1 review

Summary of findings: continuous cycles alternating aztreonam lysine for inhalation with tobramycin for inhalation solution vs continuous cycles alternating placebo with tobramycin

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

The review found no eligible studies for this question.

1 finding from 1 review

Summary of findings: continuous cycles alternating aztreonam lysine for inhalation with tobramycin for inhalation solution vs continuous cycles alternating placebo with tobramycin

Pulmonary exacerbations: rate of PDEs per participant year · adults and children with cystic fibrosis and a positive microbiological isolate of P aeruginosa from a respiratory tract specimen

Summary of findings: continuous cycles alternating aztreonam lysine… showed no clear difference in pulmonary exacerbations: rate of PDEs per participant year compared with continuous cycles alternating placebo with tobramycin (low certainty).

1 finding from 1 review

Summary of findings: continuous cycles alternating aztreonam lysine for inhalation with tobramycin for inhalation solution vs continuous cycles alternating placebo with tobramycin

Quality of life: respiratory domain · adults and children with cystic fibrosis and a positive microbiological isolate of P aeruginosa from a respiratory tract specimen
1 finding from 1 review

Summary of findings: continuous cycles alternating aztreonam lysine for inhalation with tobramycin for inhalation solution vs continuous cycles alternating placebo with tobramycin

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

The review found no eligible studies for this question.

1 finding from 1 review

Summary of findings: continuous cycles alternating aztreonam lysine for inhalation with tobramycin for inhalation solution vs continuous cycles alternating placebo with tobramycin

Antibiotic resistance: change from baseline in P aeruginosa CFU/g of sputum at week 24 · adults and children with cystic fibrosis and a positive microbiological isolate of P aeruginosa from a respiratory tract specimen
1 finding from 1 review

Summary of findings: continuous cycles alternating aztreonam lysine for inhalation with tobramycin for inhalation solution vs continuous cycles alternating placebo with tobramycin

Adverse events: any adverse event in the comparative phase · adults and children with cystic fibrosis and a positive microbiological isolate of P aeruginosa from a respiratory tract specimen

Summary of findings: continuous cycles alternating aztreonam lysine… showed no clear difference in adverse events: any adverse event in the comparative phase compared with continuous cycles alternating placebo with tobramycin (low certainty).

1 finding from 1 review

Summary of findings: anti‐pseudomonal antibiotics vs placebo

Antibiotic resistance: frequency of tobramycin‐resistant P aeruginosa · adults and children with cystic fibrosis and a positive microbiological isolate of P aeruginosa from a respiratory tract specimen

Summary of findings: anti‐pseudomonal antibiotics showed no clear difference in antibiotic resistance: frequency of tobramycin‐resistant P aeruginosa compared with placebo (moderate certainty).

1 finding from 1 review