Findings

What do systematic reviews say?

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Evidence found and GRADE certainty

204 questions · 205 findings · 17 reviews · Cystic Fibrosis and Genetic Disorders

No studies found 69 · 33.7%GRADE not reported 9 · 4.4%Very low 29 · 14.1%Low 36 · 17.6%Moderate 50 · 24.4%High 12 · 5.9%

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

Change in sputum P aeruginosa density at end of treatment (cfu/g) · adults and children with cystic fibrosis

Summary of findings: single showed no clear difference in change in sputum P aeruginosa density at end of treatment (cfu/g) compared with combination intravenous anti‐pseudomonal antibiotic therapy (moderate certainty).

2 findings 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

Intervention not reported

Liver dysfunction: proportion with changed aspartate aminotransferase or alanine aminotransferase levels (> 3x ULN) ‐ at all time points · children with familial hypercholesterolemia
1 finding from 1 review

Intervention not reported

Adverse events ‐ minor Infections Clinical events experienced by children during the 1 year period commencing at entry to the trial · 117 children with homozygous sickle cell (SS) disease

The intervention showed no clear difference in adverse events ‐ minor Infections Clinical events experienced by… (low certainty).

1 finding from 1 review

Inhaled tobramycin (TNS) (28 days) vs TNS (56 days)

Frequency of infective pulmonary exacerbations: number of exacerbations per patient year · 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) (28 days) changes frequency of infective pulmonary exacerbations: number of… compared with TNS (56 days) (very low certainty).

1 finding from 1 review

Inhaled tobramycin (TNS) (28 days) vs TNS (56 days)

Isolation of other micro‐organisms from the respiratory tract: number of positive cultures per patient year · adults and children with cystic fibrosis and a positive microbiological isolate of P aeruginosa from a respiratory tract specimen
1 finding from 1 review

Cycled inhaled tobramycin vs culture‐based TNS

Pulmonary exacerbations · 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 frequency of infective pulmonary exacerbations: proportion of… compared with culture‐based TNS (moderate certainty).

1 finding from 1 review

Cycled inhaled tobramycin vs culture‐based TNS

Isolation of other micro‐organisms from the respiratory tract: proportion of participants with new isolates of Stenotrophomonas maltophilia · adults and children with cystic fibrosis and a positive microbiological isolate of P aeruginosa from a respiratory tract specimen

Cycled inhaled tobramycin (TNS) showed no clear difference in isolation of other micro‐organisms from the respiratory tract:… compared with culture‐based TNS (moderate 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