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Search review questions

Each row is one question a systematic review asked: who, what, compared with what, and which outcome, with the effect it found and how certain that is. Words match anywhere in the field.

Limit to evidence status or GRADE (optional)

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

current versions
No studies 856 · 37.5%Ungraded 58 · 2.5%Very low 550 · 24.1%Low 502 · 22%Moderate 271 · 11.9%High 43 · 1.9%

Ungraded means an effect was reported without a GRADE rating; it is not a gap. No studies means the review found nothing to answer the question. The six categories are a display order, not a score.

QuestionEffectEvidenceStatus / GRADE
Summary of findings: levofloxacin for inhalation solution vs tobramycin
Adverse events: number of treatment‐related adverse events · adults and children aged over 12 with CF and P aeruginosa
Inhaled anti‐pseudomonal antibiotics for long‐term therapy in cystic fibrosis (2022) · Cochrane
no estimate extracted1 study · 282 participantsHigh
Summary of findings: anti‐pseudomonal antibiotics vs placebo
Survival: number of deaths · adults and children with CF and P aeruginosa
Inhaled anti‐pseudomonal antibiotics for long‐term therapy in cystic fibrosis (2022) · Cochrane
RR 0.17 [0.03, 1.09]2 studies · 767 participantsLow
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 (average values across the end of the 3 treatment cycles) · children and adults with CF and P aeruginosa
Inhaled anti‐pseudomonal antibiotics for long‐term therapy in cystic fibrosis (2022) · Cochrane
no estimate extracted1 study · 90 participantsLow
Summary of findings: continuous cycles alternating aztreonam lysine for inhalation with tobramycin for inhalation solution vs continuous cycles alternating placebo with tobramycin
FVC · children and adults with CF and P aeruginosa
Inhaled anti‐pseudomonal antibiotics for long‐term therapy in cystic fibrosis (2022) · Cochrane
no estimate extractedNRNo studies
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 · children and adults with CF and P aeruginosa
Inhaled anti‐pseudomonal antibiotics for long‐term therapy in cystic fibrosis (2022) · Cochrane
RR 0.71 [0.43, 1.18]1 study · 90 participantsLow
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: CFQ‐R respiratory symptom scores averaged from weeks 4, 12 and 20 · children and adults with CF and P aeruginosa
Inhaled anti‐pseudomonal antibiotics for long‐term therapy in cystic fibrosis (2022) · Cochrane
no estimate extracted1 study · 90 participantsLow
Summary of findings: continuous cycles alternating aztreonam lysine for inhalation with tobramycin for inhalation solution vs continuous cycles alternating placebo with tobramycin
Survival · children and adults with CF and P aeruginosa
Inhaled anti‐pseudomonal antibiotics for long‐term therapy in cystic fibrosis (2022) · Cochrane
no estimate extractedNRNo studies
Summary of findings: continuous cycles alternating aztreonam lysine for inhalation with tobramycin for inhalation solution vs continuous cycles alternating placebo with tobramycin
Antibiotic resistance: mean change from baseline in Paeruginosa sputum density (CFU/g) · children and adults with CF and P aeruginosa
Inhaled anti‐pseudomonal antibiotics for long‐term therapy in cystic fibrosis (2022) · Cochrane
no estimate extracted1 study · 87 participantsLow
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 · children and adults with CF and P aeruginosa
Inhaled anti‐pseudomonal antibiotics for long‐term therapy in cystic fibrosis (2022) · Cochrane
RR 0.97 [0.9, 1.05]1 study · 88 participantsLow
Summary of findings: anti‐pseudomonal antibiotics vs placebo
Antibiotic resistance: frequency of tobramycin‐resistant P aeruginosa · adults and children with CF and P aeruginosa
Inhaled anti‐pseudomonal antibiotics for long‐term therapy in cystic fibrosis (2022) · Cochrane
RR 1.95 [0.86, 4.42]2 studies · 672 participantsModerate
Summary of findings: anti‐pseudomonal antibiotics vs placebo
Adverse events · adults and children with CF and P aeruginosa
Inhaled anti‐pseudomonal antibiotics for long‐term therapy in cystic fibrosis (2022) · Cochrane
no estimate extracted6 studies · 1014 participantsVery low
Summary of findings: colistimethate dry powder for inhalation (Colobreathe®) vs tobramycin
FEV1 (% predicted): mean change from baseline · children and adults with CF and P aeruginosa infection
Inhaled anti‐pseudomonal antibiotics for long‐term therapy in cystic fibrosis (2022) · Cochrane
no estimate extracted1 study · 374 participantsLow
Summary of findings: colistimethate dry powder for inhalation (Colobreathe®) vs tobramycin
FVC (% predicted): mean change from baseline · children and adults with CF and P aeruginosa infection
Inhaled anti‐pseudomonal antibiotics for long‐term therapy in cystic fibrosis (2022) · Cochrane
no estimate extracted1 study · 374 participantsLow
Summary of findings ‐ Hydroxyurea vs placebo
Pain alterationa · adults and children with sickle cell disease
Hydroxyurea (hydroxycarbamide) for sickle cell disease (2022) · Cochrane
no estimate extracted5 studies · 784 participantsModerate
Summary of findings ‐ Hydroxyurea and phlebotomy vs transfusion and chelation
Death during the study (all deaths) · adults and children with sickle cell disease and an increased risk of stroke
Hydroxyurea (hydroxycarbamide) for sickle cell disease (2022) · Cochrane
RR 0.99 [0.06, 15.42]2 studies · 254 participantsLow
Summary of findings ‐ Hydroxyurea and phlebotomy vs transfusion and chelation
Measures of HbF (%) · adults and children with sickle cell disease and an increased risk of stroke
Hydroxyurea (hydroxycarbamide) for sickle cell disease (2022) · Cochrane
no estimate extracted2 studies · 254 participantsModerate
Summary of findings ‐ Hydroxyurea and phlebotomy vs transfusion and chelation
Measures of ANC · adults and children with sickle cell disease and an increased risk of stroke
Hydroxyurea (hydroxycarbamide) for sickle cell disease (2022) · Cochrane
no estimate extracted2 studies · 254 participantsModerate
Summary of findings ‐ Hydroxyurea and phlebotomy vs transfusion and chelation
Quality of life · adults and children with sickle cell disease and an increased risk of stroke
Hydroxyurea (hydroxycarbamide) for sickle cell disease (2022) · Cochrane
no estimate extractedNRNo studies
Summary of findings ‐ Hydroxyurea and phlebotomy vs transfusion and chelation
Adverse events or toxicity: differences in rates of specific adverse events · adults and children with sickle cell disease and an increased risk of stroke
Hydroxyurea (hydroxycarbamide) for sickle cell disease (2022) · Cochrane
no estimate extracted2 studies · 254 participantsLow
Summary of findings ‐ Hydroxyurea vs observation
Pain alteration · adults and children with sickle cell disease and an increased risk of stroke
Hydroxyurea (hydroxycarbamide) for sickle cell disease (2022) · Cochrane
no estimate extractedNRNo studies
Summary of findings ‐ Hydroxyurea vs observation
Life‐threatening illness · adults and children with sickle cell disease and an increased risk of stroke
Hydroxyurea (hydroxycarbamide) for sickle cell disease (2022) · Cochrane
no estimate extracted1 study · 22 participantsVery low
Summary of findings ‐ Hydroxyurea vs observation
Death during the study · adults and children with sickle cell disease and an increased risk of stroke
Hydroxyurea (hydroxycarbamide) for sickle cell disease (2022) · Cochrane
no estimate extracted1 study · 22 participantsVery low
Summary of findings ‐ Hydroxyurea vs observation
Measures of HbF · adults and children with sickle cell disease and an increased risk of stroke
Hydroxyurea (hydroxycarbamide) for sickle cell disease (2022) · Cochrane
no estimate extracted1 study · 22 participantsVery low
Summary of findings ‐ Hydroxyurea vs observation
Measures of ANC · adults and children with sickle cell disease and an increased risk of stroke
Hydroxyurea (hydroxycarbamide) for sickle cell disease (2022) · Cochrane
no estimate extracted1 study · 22 participantsVery low
Summary of findings ‐ Hydroxyurea vs placebo
Life‐threatening illness · adults and children with sickle cell disease
Hydroxyurea (hydroxycarbamide) for sickle cell disease (2022) · Cochrane
no estimate extracted4 studies · 759 participantsModerate
Summary of findings ‐ Hydroxyurea vs observation
Quality of life · adults and children with sickle cell disease and an increased risk of stroke
Hydroxyurea (hydroxycarbamide) for sickle cell disease (2022) · Cochrane
no estimate extractedNRNo studies
Summary of findings ‐ Hydroxyurea vs observation
Adverse events or toxicity: differences in rates of specific adverse events · adults and children with sickle cell disease and an increased risk of stroke
Hydroxyurea (hydroxycarbamide) for sickle cell disease (2022) · Cochrane
no estimate extracted1 study · 22 participantsVery low
Summary of findings ‐ Hydroxyurea vs no hydroxyurea
Pain alteration · adults and children with sickle cell disease
Hydroxyurea (hydroxycarbamide) for sickle cell disease (2022) · Cochrane
no estimate extractedNRNo studies
Summary of findings ‐ Hydroxyurea vs no hydroxyurea
Life‐threatening illness · adults and children with sickle cell disease
Hydroxyurea (hydroxycarbamide) for sickle cell disease (2022) · Cochrane
no estimate extractedNRNo studies
Summary of findings ‐ Hydroxyurea vs no hydroxyurea
Death during the study · adults and children with sickle cell disease
Hydroxyurea (hydroxycarbamide) for sickle cell disease (2022) · Cochrane
no estimate extracted1 study · 44 participantsVery low
Summary of findings ‐ Hydroxyurea vs no hydroxyurea
Measures of HbF · adults and children with sickle cell disease
Hydroxyurea (hydroxycarbamide) for sickle cell disease (2022) · Cochrane
no estimate extracted1 study · 44 participantsVery low
Summary of findings ‐ Hydroxyurea vs no hydroxyurea
Measures of ANC · adults and children with sickle cell disease
Hydroxyurea (hydroxycarbamide) for sickle cell disease (2022) · Cochrane
no estimate extracted1 study · 44 participantsVery low
Summary of findings ‐ Hydroxyurea vs no hydroxyurea
Quality of life · adults and children with sickle cell disease
Hydroxyurea (hydroxycarbamide) for sickle cell disease (2022) · Cochrane
no estimate extractedNRNo studies
Summary of findings ‐ Hydroxyurea vs no hydroxyurea
Adverse events or toxicity · adults and children with sickle cell disease
Hydroxyurea (hydroxycarbamide) for sickle cell disease (2022) · Cochrane
no estimate extracted1 study · 44 participantsVery low
Summary of findings ‐ Hydroxyurea vs placebo
Death during the study (all deaths) · adults and children with sickle cell disease
Hydroxyurea (hydroxycarbamide) for sickle cell disease (2022) · Cochrane
RR 0.65 [0.19, 2.27]5 studies · 784 participantsModerate
Summary of findings ‐ Hydroxyurea vs placebo
Measures of HbF (%) · adults and children with sickle cell disease
Hydroxyurea (hydroxycarbamide) for sickle cell disease (2022) · Cochrane
no estimate extracted5 studies · 784 participantsLow
Summary of findings ‐ Hydroxyurea vs placebo
Measures of ANC · adults and children with sickle cell disease
Hydroxyurea (hydroxycarbamide) for sickle cell disease (2022) · Cochrane
no estimate extracted4 studies · 724 participantsModerate
Summary of findings ‐ Hydroxyurea vs placebo
Quality of life: 'Health Status Survey', the 'Profile of Mood States' and the 'Ladder of Life' · adults and children with sickle cell disease
Hydroxyurea (hydroxycarbamide) for sickle cell disease (2022) · Cochrane
no estimate extracted1 study · 277 participantsLow
Summary of findings ‐ Hydroxyurea vs placebo
Adverse events or toxicity: differences in rates of specific adverse events · adults and children with sickle cell disease
Hydroxyurea (hydroxycarbamide) for sickle cell disease (2022) · Cochrane
no estimate extracted5 studies · 784 participantsLow
Summary of findings ‐ Hydroxyurea and phlebotomy vs transfusion and chelation
Pain alteration: proportion experiencing serious VOC or sickle‐related pain events · adults and children with sickle cell disease and an increased risk of stroke
Hydroxyurea (hydroxycarbamide) for sickle cell disease (2022) · Cochrane
RR 3.37 [1.59, 7.11]2 studies · 254 participantsLow
Summary of findings ‐ Hydroxyurea and phlebotomy vs transfusion and chelation
Life‐threatening illness · adults and children with sickle cell disease and an increased risk of stroke
Hydroxyurea (hydroxycarbamide) for sickle cell disease (2022) · Cochrane
no estimate extracted2 studies · 254 participantsModerate
Physical activity vs no physical activity
Exercise capacity: change in VO2 peak (mL/min per kg bodyweight) Active intervention: > 6 months · adults and children with cystic fibrosis
Physical activity and exercise training in cystic fibrosis (2022) · Cochrane
no estimate extracted6 studies · 348 participantsModerate
Physical activity vs no physical activity
FEV1 % predicted (change from baseline) Active intervention: > 6 months · adults and children with cystic fibrosis
Physical activity and exercise training in cystic fibrosis (2022) · Cochrane
no estimate extracted6 studies · 367 participantsLow
Physical activity vs no physical activity
HRQoL: change in CFQ‐R physical functioning domain score Active intervention: > 6 months · adults and children with cystic fibrosis
Physical activity and exercise training in cystic fibrosis (2022) · Cochrane
no estimate extracted4 studies · 247 participantsLow
Physical activity vs no physical activity
HRQoL: change in CFQ‐R respiratory symptoms domain score Active intervention: > 6 months · adults and children with cystic fibrosis
Physical activity and exercise training in cystic fibrosis (2022) · Cochrane
no estimate extracted4 studies · 251 participantsLow
Physical activity vs no physical activity
Pulmonary exacerbations: number of exacerbations occurring in the study period Active intervention: 12 months · adults and children with cystic fibrosis
Physical activity and exercise training in cystic fibrosis (2022) · Cochrane
no estimate extracted1 study · 117 participantsHigh
Physical activity vs no physical activity
Diabetic control: change in blood glucose levels at rest, at 60 and 120 minutes after a glucose ingestion (mmol/L) Active intervention: 9 months · adults and children with cystic fibrosis
Physical activity and exercise training in cystic fibrosis (2022) · Cochrane
no estimate extracted1 study · 91 participantsModerate
Physical activity vs no physical activity
Adverse events: number of adverse events Active intervention: 12 months · adults and children with cystic fibrosis
Physical activity and exercise training in cystic fibrosis (2022) · Cochrane
no estimate extracted2 studies · 156 participantsLow
Appetite stimulants vs placebo
Change in weight (kg) · people with cystic fibrosis
Appetite stimulants for people with cystic fibrosis (2022) · Cochrane
no estimate extracted2 studies · 42 participantsLow
Appetite stimulants vs placebo
Change in weight (kg) · people with cystic fibrosis
Appetite stimulants for people with cystic fibrosis (2022) · Cochrane
no estimate extracted1 study · 17 participantsLow