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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.
2,280 questions in 114 reviews · Cystic Fibrosis and Genetic Disorders
current versionsNo 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.
| Question | Effect | Evidence | Status / GRADE |
|---|---|---|---|
Summary of findings: oral antioxidant combination vs control Nutritional status: change from baseline in BMI · children with cystic fibrosis Antioxidant supplementation for lung disease in cystic fibrosis (2019) · Cochrane | no estimate extracted | NR | No studies |
Summary of findings: oral antioxidant combination vs control Pulmonary exacerbations: mean time to next exacerbation · children with cystic fibrosis Antioxidant supplementation for lung disease in cystic fibrosis (2019) · Cochrane | no estimate extracted | NR | No studies |
Summary of findings: oral antioxidant combination vs control Adverse events · children with cystic fibrosis Antioxidant supplementation for lung disease in cystic fibrosis (2019) · Cochrane | no estimate extracted | NR | No studies |
Summary of findings: oral antioxidant mixed supplement vs control Lung function: FEV1 % predicted mean change from baseline · adults and children with cystic fibrosis Antioxidant supplementation for lung disease in cystic fibrosis (2019) · Cochrane | no estimate extracted | NR | No studies |
Summary of findings: oral antioxidant mixed supplement vs control Lung function: FEV1 % predicted mean change from baseline · adults and children with cystic fibrosis Antioxidant supplementation for lung disease in cystic fibrosis (2019) · Cochrane | no estimate extracted | NR | No studies |
Summary of findings: oral antioxidant mixed supplement vs control Quality of life: Quality of Well Being score change from baseline · adults and children with cystic fibrosis Antioxidant supplementation for lung disease in cystic fibrosis (2019) · Cochrane | no estimate extracted | NR | No studies |
Summary of findings: oral antioxidant mixed supplement vs control Nutritional status: change from baseline in BMI · adults and children with cystic fibrosis Antioxidant supplementation for lung disease in cystic fibrosis (2019) · Cochrane | no estimate extracted | NR | No studies |
Summary of findings: oral antioxidant mixed supplement vs control Pulmonary exacerbations: mean time to next exacerbation · adults and children with cystic fibrosis Antioxidant supplementation for lung disease in cystic fibrosis (2019) · Cochrane | no estimate extracted | 1 study · 69 participants | Low |
Summary of findings: oral antioxidants (NAC/GSH) vs placebo Quality of life: change in mean CFQ‐R score from baseline · adults and children with cystic fibrosis Antioxidant supplementation for lung disease in cystic fibrosis (2019) · Cochrane | no estimate extracted | 1 study · 61 participants | Moderate |
Summary of findings: oral antioxidant mixed supplement vs control Adverse events · adults and children with cystic fibrosis Antioxidant supplementation for lung disease in cystic fibrosis (2019) · Cochrane | no estimate extracted | 1 study · 69 participants | Low |
Summary of findings: inhaled antioxidants vs placebo Lung function: FEV1 % predicted mean change from baseline · children and adults with cystic fibrosis Antioxidant supplementation for lung disease in cystic fibrosis (2019) · Cochrane | no estimate extracted | 3 studies · 258 participants | Moderate |
Summary of findings: inhaled antioxidants vs placebo Lung function: FEV1 % predicted mean change from baseline · children and adults with cystic fibrosis Antioxidant supplementation for lung disease in cystic fibrosis (2019) · Cochrane | no estimate extracted | 3 studies · 258 participants | Moderate |
Summary of findings: inhaled antioxidants vs placebo Quality of life: change in CFQoL score from baseline · children and adults with cystic fibrosis Antioxidant supplementation for lung disease in cystic fibrosis (2019) · Cochrane | no estimate extracted | 1 study · 153 participants | Moderate |
Summary of findings: inhaled antioxidants vs placebo Nutritional status: change from baseline in BMI · children and adults with cystic fibrosis Antioxidant supplementation for lung disease in cystic fibrosis (2019) · Cochrane | no estimate extracted | NR | No studies |
Summary of findings: inhaled antioxidants vs placebo Pulmonary exacerbations: mean time to next exacerbation · children and adults with cystic fibrosis Antioxidant supplementation for lung disease in cystic fibrosis (2019) · Cochrane | no estimate extracted | NR | No studies |
Summary of findings: inhaled antioxidants vs placebo Adverse events · children and adults with cystic fibrosis Antioxidant supplementation for lung disease in cystic fibrosis (2019) · Cochrane | no estimate extracted | 3 studies · 223 participants | Low |
Summary of findings: oral antioxidants (NAC/GSH) vs placebo Nutritional status: change from baseline in BMI · adults and children with cystic fibrosis Antioxidant supplementation for lung disease in cystic fibrosis (2019) · Cochrane | no estimate extracted | 1 study · 62 participants | Moderate |
Summary of findings: oral antioxidants (NAC/GSH) vs placebo Pulmonary exacerbations: mean time to next exacerbation · adults and children with cystic fibrosis Antioxidant supplementation for lung disease in cystic fibrosis (2019) · Cochrane | no estimate extracted | NR | No studies |
Summary of findings: oral antioxidants (NAC/GSH) vs placebo Adverse events · adults and children with cystic fibrosis Antioxidant supplementation for lung disease in cystic fibrosis (2019) · Cochrane | OR 2.92 [0.11, 74.05] | 1 study · 70 participants | Moderate |
Summary of findings: oral vitamin E supplementation vs placebo or no treatment Lung function: FEV1 % predicted mean change from baseline · children and adults with cystic fibrosis Antioxidant supplementation for lung disease in cystic fibrosis (2019) · Cochrane | no estimate extracted | NR | No studies |
Summary of findings: oral vitamin E supplementation vs placebo or no treatment Lung function: FEV1 % predicted mean change from baseline · children and adults with cystic fibrosis Antioxidant supplementation for lung disease in cystic fibrosis (2019) · Cochrane | no estimate extracted | NR | No studies |
Summary of findings: oral vitamin E supplementation vs placebo or no treatment Quality of life · children and adults with cystic fibrosis Antioxidant supplementation for lung disease in cystic fibrosis (2019) · Cochrane | no estimate extracted | NR | No studies |
Intervention NR Change in urinary GAG excretion from baseline · adults and children with mucopolysaccharidosis I Enzyme replacement therapy with laronidase (Aldurazyme®) for treating mucopolysaccharidosis type I (2019) · Cochrane | no estimate extracted | 1 study · 43 participants | Low |
Intervention NR Mean percentage change from baseline in FVC (% predicted) · adults and children with mucopolysaccharidosis I Enzyme replacement therapy with laronidase (Aldurazyme®) for treating mucopolysaccharidosis type I (2019) · Cochrane | no estimate extracted | 1 study · 45 participants | Low |
Intervention NR Mean change from baseline in 6MWT (metres) · adults and children with mucopolysaccharidosis I Enzyme replacement therapy with laronidase (Aldurazyme®) for treating mucopolysaccharidosis type I (2019) · Cochrane | no estimate extracted | 1 study · 45 participants | Low |
Intervention NR Infusion‐related reactions (number of participants with one or more reaction) · adults and children with mucopolysaccharidosis I Enzyme replacement therapy with laronidase (Aldurazyme®) for treating mucopolysaccharidosis type I (2019) · Cochrane | OR 0.51 [0.15, 1.71] | 1 study · 45 participants | Low |
Intervention NR Antibody production: number of participants developing IgG antibodies · adults and children with mucopolysaccharidosis I Enzyme replacement therapy with laronidase (Aldurazyme®) for treating mucopolysaccharidosis type I (2019) · Cochrane | OR 385.4 [17.47, 8500.51] | 1 study · 45 participants | Very low |
Intervention NR Quality of life · adults and children with mucopolysaccharidosis I Enzyme replacement therapy with laronidase (Aldurazyme®) for treating mucopolysaccharidosis type I (2019) · Cochrane | no estimate extracted | 1 study · 45 participants | Low |
Intervention NR Mortality: number of deaths · adults and children with mucopolysaccharidosis I Enzyme replacement therapy with laronidase (Aldurazyme®) for treating mucopolysaccharidosis type I (2019) · Cochrane | no estimate extracted | 1 study · 45 participants | Low |
Summary of findings ‐ ivacaftor vs placebo Survival · adults and children with cystic fibrosis and with the F508del CFTR mutation Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis (2019) · Cochrane | no estimate extracted | 1 study · 140 participants | Ungraded |
Summary of findings ‐ ivacaftor vs placebo QoL (CFQ‐R) Respiratory domain · adults and children with cystic fibrosis and with at least one G551D CFTR mutation Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis (2019) · Cochrane | no estimate extracted | 3 studies · 222 participants | Moderate |
Summary of findings ‐ ivacaftor vs placebo FEV1 % predicted Relative change from baseline · adults and children with cystic fibrosis and with at least one G551D CFTR mutation Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis (2019) · Cochrane | no estimate extracted | 3 studies · 232 participants | Moderate |
Summary of findings ‐ ivacaftor vs placebo FEV1 L and % predicted Absolute change from baseline · adults and children with cystic fibrosis and with at least one G551D CFTR mutation Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis (2019) · Cochrane | no estimate extracted | 3 studies · 232 participants | Low |
Summary of findings ‐ ivacaftor vs placebo Adverse events · adults and children with cystic fibrosis and with at least one G551D CFTR mutation Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis (2019) · Cochrane | no estimate extracted | 3 studies · 232 participants | Low |
Summary of findings ‐ ivacaftor vs placebo Time to first pulmonary exacerbation · adults and children with cystic fibrosis and with at least one G551D CFTR mutation Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis (2019) · Cochrane | HR 0.46 [0.29, 0.73] | 1 study · 161 participants | Moderate |
Summary of findings ‐ ivacaftor vs placebo Survival · adults and children with cystic fibrosis and with at least one R117H CFTR mutation Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis (2019) · Cochrane | no estimate extracted | 1 study · 69 participants | Ungraded |
Summary of findings ‐ ivacaftor vs placebo QoL Total score · adults and children with cystic fibrosis and with at least one R117H CFTR mutation Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis (2019) · Cochrane | no estimate extracted | NR | No studies |
Summary of findings ‐ ivacaftor vs placebo QoL (CFQ‐R) Respiratory domain · adults and children with cystic fibrosis and with at least one R117H CFTR mutation Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis (2019) · Cochrane | no estimate extracted | 1 study · 69 participants | Moderate |
Summary of findings ‐ ivacaftor vs placebo FEV1 % predicted Relative change from baseline · adults and children with cystic fibrosis and with at least one R117H CFTR mutation Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis (2019) · Cochrane | no estimate extracted | 1 study · 69 participants | Moderate |
Summary of findings ‐ ivacaftor vs placebo FEV1 L and % predicted Absolute change from baseline · adults and children with cystic fibrosis and with at least one R117H CFTR mutation Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis (2019) · Cochrane | no estimate extracted | 1 study · 69 participants | Moderate |
Summary of findings ‐ ivacaftor vs placebo QoL Total score · adults and children with cystic fibrosis and with the F508del CFTR mutation Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis (2019) · Cochrane | no estimate extracted | NR | No studies |
Summary of findings ‐ ivacaftor vs placebo Adverse events · adults and children with cystic fibrosis and with at least one R117H CFTR mutation Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis (2019) · Cochrane | no estimate extracted | 1 study · 69 participants | Low |
Summary of findings ‐ ivacaftor vs placebo Time to first pulmonary exacerbation · adults and children with cystic fibrosis and with at least one R117H CFTR mutation Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis (2019) · Cochrane | HR 0.93 [0.42, 2.08] | 1 study · 69 participants | Moderate |
Summary of findings ‐ ivacaftor vs placebo QoL (CFQ‐R) Respiratory domain · adults and children with cystic fibrosis and with the F508del CFTR mutation Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis (2019) · Cochrane | no estimate extracted | 1 study · 140 participants | Moderate |
Summary of findings ‐ ivacaftor vs placebo FEV1 % predicted Relative change from baseline · adults and children with cystic fibrosis and with the F508del CFTR mutation Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis (2019) · Cochrane | no estimate extracted | 1 study · 140 participants | Moderate |
Summary of findings ‐ ivacaftor vs placebo FEV1 % predicted Absolute change from baseline · adults and children with cystic fibrosis and with the F508del CFTR mutation Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis (2019) · Cochrane | no estimate extracted | 1 study · 140 participants | Moderate |
Summary of findings ‐ ivacaftor vs placebo Adverse events · adults and children with cystic fibrosis and with the F508del CFTR mutation Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis (2019) · Cochrane | no estimate extracted | 1 study · 140 participants | Low |
Summary of findings ‐ ivacaftor vs placebo Time to first pulmonary exacerbation · adults and children with cystic fibrosis and with the F508del CFTR mutation Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis (2019) · Cochrane | no estimate extracted | NR | No studies |
Summary of findings ‐ ivacaftor vs placebo Survival · adults and children with cystic fibrosis and with at least one G551D CFTR mutation Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis (2019) · Cochrane | no estimate extracted | 3 studies · 238 participants | Ungraded |
Summary of findings ‐ ivacaftor vs placebo QoL Total score · adults and children with cystic fibrosis and with at least one G551D CFTR mutation Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis (2019) · Cochrane | no estimate extracted | NR | No studies |