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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
Probiotics vs placebo
Weight (z score) · children and adults with CF
Probiotics for people with cystic fibrosis (2020) · Cochrane
no estimate extracted2 studies · 91 participantsLow
Probiotics vs placebo
Lung function (FEV1 % predicted) · children and adults with CF
Probiotics for people with cystic fibrosis (2020) · Cochrane
no estimate extracted5 studies · 284 participantsLow
Probiotics vs placebo
Hospitalisations (all causes) · children and adults with CF
Probiotics for people with cystic fibrosis (2020) · Cochrane
no estimate extracted2 studies · 115 participantsLow
Probiotics vs placebo
HRQoL (PedsQLTM 4.0 SF 15 (Scale from: 0 to 100)) · children and adults with CF
Probiotics for people with cystic fibrosis (2020) · Cochrane
no estimate extracted1 study · 37 participantsLow
Intervention vs usual care
Change in quality of life · postural abnormalities in people with cystic fibrosis
Physical therapies for postural abnormalities in people with cystic fibrosis (2020) · Cochrane
no estimate extracted2 studies · 50 participantsVery low
Intervention vs usual care
Change in pain (mm) · postural abnormalities in people with cystic fibrosis
Physical therapies for postural abnormalities in people with cystic fibrosis (2020) · Cochrane
no estimate extracted2 studies · 50 participantsVery low
Intervention vs usual care
Change in trunk deformity (cm) · postural abnormalities in people with cystic fibrosis
Physical therapies for postural abnormalities in people with cystic fibrosis (2020) · Cochrane
no estimate extracted2 studies · 50 participantsVery low
Intervention vs usual care
Change in FEV1 (L) · postural abnormalities in people with cystic fibrosis
Physical therapies for postural abnormalities in people with cystic fibrosis (2020) · Cochrane
no estimate extracted2 studies · 50 participantsVery low
Intervention vs usual care
Change in FVC (L) · postural abnormalities in people with cystic fibrosis
Physical therapies for postural abnormalities in people with cystic fibrosis (2020) · Cochrane
no estimate extracted2 studies · 50 participantsVery low
Intervention vs usual care
Change in ERV (L) · postural abnormalities in people with cystic fibrosis
Physical therapies for postural abnormalities in people with cystic fibrosis (2020) · Cochrane
no estimate extractedNRNo studies
Intervention vs usual care
Change in TLC (L) · postural abnormalities in people with cystic fibrosis
Physical therapies for postural abnormalities in people with cystic fibrosis (2020) · Cochrane
no estimate extractedNRNo studies
Antifibrinolytic therapy (tranexamic acid or epsilon aminocaproic acid) vs Placebo, no intervention or usual care (with or without placebo)
Number of minor postoperative bleeding episodes · People with haemophilia (A or B) undergoing minor oral surgery or dental extractions
Antifibrinolytic therapy for preventing oral bleeding in patients with haemophilia or Von Willebrand disease… (2019) · Cochrane
This outcome was not reported in either of the trials.0 studiesNo studies
Antifibrinolytic therapy (tranexamic acid or epsilon aminocaproic acid) vs Placebo, no intervention or usual care (with or without placebo)
Outcome NR · People with Von Willebrand disease undergoing minor oral surgery or dental extractions
Antifibrinolytic therapy for preventing oral bleeding in patients with haemophilia or Von Willebrand disease… (2019) · Cochrane
No randomised or quasi-randomised controlled trials in people with Von Willebrand disease met the inclusion criteria; no eligible VWD trials were identified.0 studiesNo studies
Antifibrinolytic therapy (tranexamic acid or epsilon aminocaproic acid) vs Placebo, no intervention or usual care (with or without placebo)
Major bleeding, requiring transfusion of packed red blood cells · People with haemophilia (A or B) undergoing minor oral surgery or dental extractions
Antifibrinolytic therapy for preventing oral bleeding in patients with haemophilia or Von Willebrand disease… (2019) · Cochrane
Only reported in the Walsh (EACA) trial: in none of the 31 included participants was blood transfusion required.1 study · 31 participantsUngraded
Antifibrinolytic therapy (tranexamic acid or epsilon aminocaproic acid) vs Placebo, no intervention or usual care (with or without placebo)
Change in haemoglobin level from baseline · People with haemophilia (A or B) undergoing minor oral surgery or dental extractions
Antifibrinolytic therapy for preventing oral bleeding in patients with haemophilia or Von Willebrand disease… (2019) · Cochrane
Mean fall in haemoglobin. Forbes (TXA): 1.4 g/100 mL (placebo) vs 0.3 g/100 mL (TXA). Walsh (EACA): 6.3% (placebo, both centres) vs 3.9% and 3.7% (EACA, centres 1 and 2); differences not significant. Reported in…2 studies · 59 participantsUngraded
Antifibrinolytic therapy (tranexamic acid or epsilon aminocaproic acid) vs Placebo, no intervention or usual care (with or without placebo)
Bleeding duration · People with haemophilia (A or B) undergoing minor oral surgery or dental extractions
Antifibrinolytic therapy for preventing oral bleeding in patients with haemophilia or Von Willebrand disease… (2019) · Cochrane
This outcome was not reported in either of the trials.0 studiesNo studies
Antifibrinolytic therapy (tranexamic acid or epsilon aminocaproic acid) vs Placebo, no intervention or usual care (with or without placebo)
Need for and dose of clotting factor concentrates · People with haemophilia (A or B) undergoing minor oral surgery or dental extractions
Antifibrinolytic therapy for preventing oral bleeding in patients with haemophilia or Von Willebrand disease… (2019) · Cochrane
Reported in both trials in different ways. Forbes (TXA): mean units of replacement therapy per root extracted 617 IU (range 0 to 15,800) in 11 placebo participants versus 30 IU and 65 IU per root in 2 TXA participants…2 studies · 59 participantsUngraded
Antifibrinolytic therapy (tranexamic acid or epsilon aminocaproic acid) vs Placebo, no intervention or usual care (with or without placebo)
Any postoperative complication except bleeding (such as wound infection) · People with haemophilia (A or B) undergoing minor oral surgery or dental extractions
Antifibrinolytic therapy for preventing oral bleeding in patients with haemophilia or Von Willebrand disease… (2019) · Cochrane
This outcome was not reported in either of the trials.0 studiesNo studies
Antifibrinolytic therapy (tranexamic acid or epsilon aminocaproic acid) vs Placebo, no intervention or usual care (with or without placebo)
Number of postoperative bleeding episodes requiring intervention · People with haemophilia (A or B) undergoing minor oral surgery or dental extractions · Up to 10 days postoperative
Antifibrinolytic therapy for preventing oral bleeding in patients with haemophilia or Von Willebrand disease… (2019) · Cochrane
RD -0.57 [-0.76, -0.37]2 studies · 59 participantsModerate
Antifibrinolytic therapy (tranexamic acid or epsilon aminocaproic acid) vs Placebo, no intervention or usual care (with or without placebo)
Amount of postoperative blood loss (mL) · People with haemophilia (A or B) undergoing minor oral surgery or dental extractions
Antifibrinolytic therapy for preventing oral bleeding in patients with haemophilia or Von Willebrand disease… (2019) · Cochrane
Only reported in the Forbes (TXA) trial: over five-day follow-up, mean blood loss per participant 84.1 mL (range 4 to 323) in the placebo group (n=14) versus 61.2 mL (range 1 to 749) in the TXA group (n=14), P = 0.02.1 study · 28 participantsUngraded
Summary of findings: non‐steroidal anti‐inflammatory drugs vs placebo
Annual rate of change in % predicted FEV1 All participants · adults and children with cystic fibrosis
Oral non‐steroidal anti‐inflammatory drug therapy for lung disease in cystic fibrosis (2019) · Cochrane
no estimate extracted2 studies · 226 participantsModerate
Summary of findings: non‐steroidal anti‐inflammatory drugs vs placebo
Annual rate of change in % predicted FEV1 Subgroup: under 13 years of age · adults and children with cystic fibrosis
Oral non‐steroidal anti‐inflammatory drug therapy for lung disease in cystic fibrosis (2019) · Cochrane
no estimate extracted2 studies · 147 participantsModerate
Summary of findings: non‐steroidal anti‐inflammatory drugs vs placebo
Annual rate of change in % predicted FEV1 Subgroup: over 13 years of age · adults and children with cystic fibrosis
Oral non‐steroidal anti‐inflammatory drug therapy for lung disease in cystic fibrosis (2019) · Cochrane
no estimate extracted2 studies · 79 participantsModerate
Summary of findings: non‐steroidal anti‐inflammatory drugs vs placebo
Annual rate of change in % predicted FVC All participants · adults and children with cystic fibrosis
Oral non‐steroidal anti‐inflammatory drug therapy for lung disease in cystic fibrosis (2019) · Cochrane
no estimate extracted2 studies · 226 participantsModerate
Summary of findings: non‐steroidal anti‐inflammatory drugs vs placebo
Annual rate of change in % predicted FVC Subgroup: Under 13 years of age · adults and children with cystic fibrosis
Oral non‐steroidal anti‐inflammatory drug therapy for lung disease in cystic fibrosis (2019) · Cochrane
no estimate extracted2 studies · 147 participantsModerate
Summary of findings: non‐steroidal anti‐inflammatory drugs vs placebo
Annual rate of change in % predicted FVC Subgroup: Over 13 years of age · adults and children with cystic fibrosis
Oral non‐steroidal anti‐inflammatory drug therapy for lung disease in cystic fibrosis (2019) · Cochrane
no estimate extracted2 studies · 79 participantsModerate
Summary of findings: non‐steroidal anti‐inflammatory drugs vs placebo
Proportion with at least 1 hospital admission · adults and children with cystic fibrosis
Oral non‐steroidal anti‐inflammatory drug therapy for lung disease in cystic fibrosis (2019) · Cochrane
OR 0.61 [0.37, 1.01]4 studies · 286 participantsModerate
Summary of findings: inhaled corticosteroids vs placebo
Lung function: FEV1 % predicted (change from baseline) · children and adults with cystic fibrosis
Inhaled corticosteroids for cystic fibrosis (2019) · Cochrane
no estimate extracted3 studies · 250 participantsLow
Summary of findings: inhaled corticosteroids vs placebo
Lung function: FVC % predicted (change from baseline) · children and adults with cystic fibrosis
Inhaled corticosteroids for cystic fibrosis (2019) · Cochrane
no estimate extracted3 studies · 250 participantsLow
Summary of findings: inhaled corticosteroids vs placebo
BHR: change in PC20 values · children and adults with cystic fibrosis
Inhaled corticosteroids for cystic fibrosis (2019) · Cochrane
no estimate extracted3 studies · 100 participantsVery low
Summary of findings: inhaled corticosteroids vs placebo
Improvement in exercise tolerance · children and adults with cystic fibrosis
Inhaled corticosteroids for cystic fibrosis (2019) · Cochrane
no estimate extracted1 study · 12 participantsVery low
Summary of findings: inhaled corticosteroids vs placebo
Respiratory exacerbations: number of days on intravenous antibiotics for respiratory exacerbations · children and adults with cystic fibrosis
Inhaled corticosteroids for cystic fibrosis (2019) · Cochrane
no estimate extracted1 study · 17 participantsVery low
Summary of findings: inhaled corticosteroids vs placebo
Quality of life: change in well‐being score · children and adults with cystic fibrosis
Inhaled corticosteroids for cystic fibrosis (2019) · Cochrane
no estimate extracted1 study · 23 participantsLow
Summary of findings: inhaled corticosteroids vs placebo
Adverse effects · children and adults with cystic fibrosis
Inhaled corticosteroids for cystic fibrosis (2019) · Cochrane
no estimate extracted5 studies · 295 participantsLow
Intervention NR
Lung function Mean percentage change in FEV1 · adults and children with cystic fibrosis
Once‐daily versus multiple‐daily dosing with intravenous aminoglycosides for cystic fibrosis (2019) · Cochrane
no estimate extracted3 studies · 289 participantsModerate
Intervention NR
Lung function Mean percentage change in FVC (% predicted) · adults and children with cystic fibrosis
Once‐daily versus multiple‐daily dosing with intravenous aminoglycosides for cystic fibrosis (2019) · Cochrane
no estimate extracted2 studies · 70 participantsLow
Intervention NR
Nutritional status BMI · adults and children with cystic fibrosis
Once‐daily versus multiple‐daily dosing with intravenous aminoglycosides for cystic fibrosis (2019) · Cochrane
no estimate extracted1 study · 41 participantsLow
Intervention NR
Time to first exacerbation requiring intravenous antibiotics · adults and children with cystic fibrosis
Once‐daily versus multiple‐daily dosing with intravenous aminoglycosides for cystic fibrosis (2019) · Cochrane
no estimate extracted1 study · 113 participantsModerate
Intervention NR
Antibiotic resistance patterns following treatment · adults and children with cystic fibrosis
Once‐daily versus multiple‐daily dosing with intravenous aminoglycosides for cystic fibrosis (2019) · Cochrane
no estimate extractedNRNo studies
Intervention NR
Ototoxicity An increase in auditory threshold of 20 dB or more over any frequency range · adults and children with cystic fibrosis
Once‐daily versus multiple‐daily dosing with intravenous aminoglycosides for cystic fibrosis (2019) · Cochrane
RR 0.56 [0.04, 7.96]3 studies · 266 participantsLow
Intervention NR
Nephrotoxicity The percentage change in creatinine over baseline · adults and children with cystic fibrosis
Once‐daily versus multiple‐daily dosing with intravenous aminoglycosides for cystic fibrosis (2019) · Cochrane
no estimate extracted3 studies · 245 participantsModerate
Summary of findings ‐ PEP vs PDPV
FEV1: change from baseline (% predicted) · adults and children with CF
Positive expiratory pressure physiotherapy for airway clearance in people with cystic fibrosis (2019) · Cochrane
no estimate extracted5 studies · 155 participantsLow
Summary of findings ‐ PEP vs oscillating PEP
Direct measures of mucus clearance · adults and children with CF
Positive expiratory pressure physiotherapy for airway clearance in people with cystic fibrosis (2019) · Cochrane
no estimate extractedNRNo studies
Summary of findings ‐ PEP vs oscillating PEP
Exercise tolerance: modified shuttle test · adults and children with CF
Positive expiratory pressure physiotherapy for airway clearance in people with cystic fibrosis (2019) · Cochrane
no estimate extracted2 studies · 68 participantsModerate
Summary of findings ‐ PEP vs oscillating PEP
Well being: QWB scale. CF Short Form ‐36 and Chronic Respiratory Questionnaire · adults and children with CF
Positive expiratory pressure physiotherapy for airway clearance in people with cystic fibrosis (2019) · Cochrane
no estimate extracted2 studies · 75 participantsLow
Summary of findings ‐ PEP vs oscillating PEP
LCI · adults and children with CF
Positive expiratory pressure physiotherapy for airway clearance in people with cystic fibrosis (2019) · Cochrane
no estimate extracted1 study · 30 participantsLow
Summary of findings ‐ PEP vs oscillating PEP
Adverse events · adults and children with CF
Positive expiratory pressure physiotherapy for airway clearance in people with cystic fibrosis (2019) · Cochrane
no estimate extracted22 participantsLow
Summary of findings ‐ PEP vs HFCWO
FEV1: change from baseline (% predicted) · adults and children with CF
Positive expiratory pressure physiotherapy for airway clearance in people with cystic fibrosis (2019) · Cochrane
no estimate extracted4 studies · 174 participantsLow
Summary of findings ‐ PEP vs HFCWO
Number of respiratory exacerbations · adults and children with CF
Positive expiratory pressure physiotherapy for airway clearance in people with cystic fibrosis (2019) · Cochrane
RR 0.73 [0.55, 0.95]1 study · 107 participantsHigh
Summary of findings ‐ PEP vs HFCWO
Direct measures of mucus clearance · adults and children with CF
Positive expiratory pressure physiotherapy for airway clearance in people with cystic fibrosis (2019) · Cochrane
no estimate extractedNRNo studies