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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 |
|---|---|---|---|
Statins with cholesterol supplementation vs cholesterol supplementation only Overall survival · children and adults with Smith‐Lemli‐Opitz syndrome Statins for Smith‐Lemli‐Opitz syndrome (2022) · Cochrane | no estimate extracted | NR | No studies |
Statins with cholesterol supplementation vs cholesterol supplementation only Change in neurobehavioral manifestations · children and adults with Smith‐Lemli‐Opitz syndrome Statins for Smith‐Lemli‐Opitz syndrome (2022) · Cochrane | no estimate extracted | 1 study · 14 participants | Very low |
Statins with cholesterol supplementation vs cholesterol supplementation only Statin‐related adverse events · children and adults with Smith‐Lemli‐Opitz syndrome Statins for Smith‐Lemli‐Opitz syndrome (2022) · Cochrane | RR 0.33 [0.01, 7.68] | 1 study · 18 participants | Very low |
Statins with cholesterol supplementation vs cholesterol supplementation only Changes in growth (number of children showing change) · children and adults with Smith‐Lemli‐Opitz syndrome Statins for Smith‐Lemli‐Opitz syndrome (2022) · Cochrane | no estimate extracted | 1 study · 18 participants | Very low |
Statins with cholesterol supplementation vs cholesterol supplementation only Changes in biochemical markers plasma dehydrocholesterol levels (mM); plasma cholesterol levels (mM); plasma 7DHC levels (mM); plasma CoQ levels (uM); CSF cholesterol levels (ug/mL); CSF dehydrocholesterol levels (ug/mL) · children and adults with Smith‐Lemli‐Opitz syndrome Statins for Smith‐Lemli‐Opitz syndrome (2022) · Cochrane | no estimate extracted | 1 study · 18 participants | Low |
Statins with cholesterol supplementation vs cholesterol supplementation only QoL · children and adults with Smith‐Lemli‐Opitz syndrome Statins for Smith‐Lemli‐Opitz syndrome (2022) · Cochrane | no estimate extracted | NR | No studies |
Short‐acting inhaled beta‐2 agonists vs placebo Change from baseline in FEV1 (% predicted) · children and adults with cystic fibrosis Short‐acting inhaled bronchodilators for cystic fibrosis (2022) · Cochrane | no estimate extracted | 4 studies · 67 participants | Very low |
Short‐acting inhaled muscarinic antagonists vs placebo Number of exacerbations  · children and adults with cystic fibrosis Short‐acting inhaled bronchodilators for cystic fibrosis (2022) · Cochrane | no estimate extracted | NR | No studies |
Short‐acting inhaled muscarinic antagonists vs placebo Participant‐reported symptom score  · children and adults with cystic fibrosis Short‐acting inhaled bronchodilators for cystic fibrosis (2022) · Cochrane | no estimate extracted | NR | No studies |
Short‐acting inhaled muscarinic antagonists vs placebo Adverse events  · children and adults with cystic fibrosis Short‐acting inhaled bronchodilators for cystic fibrosis (2022) · Cochrane | no estimate extracted | 2 studies · 27 participants | Very low |
Short‐acting inhaled muscarinic antagonists vs placebo Change from baseline in FVC (% predicted) Â · children and adults with cystic fibrosis Short‐acting inhaled bronchodilators for cystic fibrosis (2022) · Cochrane | no estimate extracted | NR | No studies |
Short‐acting inhaled muscarinic antagonists vs placebo Sputum volume (mL) Â · children and adults with cystic fibrosis Short‐acting inhaled bronchodilators for cystic fibrosis (2022) · Cochrane | no estimate extracted | NR | No studies |
Short‐acting inhaled beta‐2 agonists vs short‐acting inhaled muscarinic antagonists Change from baseline in FEV1 (% predicted) Â · children and adults with cystic fibrosis Short‐acting inhaled bronchodilators for cystic fibrosis (2022) · Cochrane | no estimate extracted | NR | No studies |
Short‐acting inhaled beta‐2 agonists vs short‐acting inhaled muscarinic antagonists Quality of life  · children and adults with cystic fibrosis Short‐acting inhaled bronchodilators for cystic fibrosis (2022) · Cochrane | no estimate extracted | NR | No studies |
Short‐acting inhaled beta‐2 agonists vs short‐acting inhaled muscarinic antagonists Number of exacerbations  · children and adults with cystic fibrosis Short‐acting inhaled bronchodilators for cystic fibrosis (2022) · Cochrane | no estimate extracted | NR | No studies |
Short‐acting inhaled beta‐2 agonists vs short‐acting inhaled muscarinic antagonists Participant‐reported symptom score  · children and adults with cystic fibrosis Short‐acting inhaled bronchodilators for cystic fibrosis (2022) · Cochrane | no estimate extracted | NR | No studies |
Short‐acting inhaled beta‐2 agonists vs short‐acting inhaled muscarinic antagonists Adverse events  · children and adults with cystic fibrosis Short‐acting inhaled bronchodilators for cystic fibrosis (2022) · Cochrane | no estimate extracted | 1 study · 17 participants | Very low |
Short‐acting inhaled beta‐2 agonists vs placebo Quality of life  · children and adults with cystic fibrosis Short‐acting inhaled bronchodilators for cystic fibrosis (2022) · Cochrane | no estimate extracted | NR | No studies |
Short‐acting inhaled beta‐2 agonists vs short‐acting inhaled muscarinic antagonists Change from baseline in FVC (% predicted) Â · children and adults with cystic fibrosis Short‐acting inhaled bronchodilators for cystic fibrosis (2022) · Cochrane | no estimate extracted | NR | No studies |
Short‐acting inhaled beta‐2 agonists vs short‐acting inhaled muscarinic antagonists Sputum volume (mL) Â · children and adults with cystic fibrosis Short‐acting inhaled bronchodilators for cystic fibrosis (2022) · Cochrane | no estimate extracted | NR | No studies |
Short‐acting inhaled beta‐2 agonists vs placebo Number of exacerbations(as reported by number of hospitalisations and number of courses and days on antibiotics) · children and adults with cystic fibrosis Short‐acting inhaled bronchodilators for cystic fibrosis (2022) · Cochrane | no estimate extracted | 1 study · 21 participants | Very low |
Short‐acting inhaled beta‐2 agonists vs placebo Participant‐reported symptom score: cough, wheeze and exercise tolerance · children and adults with cystic fibrosis Short‐acting inhaled bronchodilators for cystic fibrosis (2022) · Cochrane | no estimate extracted | 1 study · 22 participants | Very low |
Short‐acting inhaled beta‐2 agonists vs placebo Adverse events  · children and adults with cystic fibrosis Short‐acting inhaled bronchodilators for cystic fibrosis (2022) · Cochrane | no estimate extracted | 3 studies · 47 participants | Very low |
Short‐acting inhaled beta‐2 agonists vs placebo Change from baseline in FVC (% predicted) · children and adults with cystic fibrosis Short‐acting inhaled bronchodilators for cystic fibrosis (2022) · Cochrane | no estimate extracted | 5 studies · 100 participants | Very low |
Short‐acting inhaled beta‐2 agonists vs placebo Sputum volume (mL) · children and adults with cystic fibrosis Short‐acting inhaled bronchodilators for cystic fibrosis (2022) · Cochrane | no estimate extracted | 1 study · 16 participants | Very low |
Short‐acting inhaled muscarinic antagonists vs placebo Change from baseline in FEV1 (% predicted) · children and adults with cystic fibrosis Short‐acting inhaled bronchodilators for cystic fibrosis (2022) · Cochrane | no estimate extracted | 1 study · 11 participants | Very low |
Short‐acting inhaled muscarinic antagonists vs placebo Quality of life  · children and adults with cystic fibrosis Short‐acting inhaled bronchodilators for cystic fibrosis (2022) · Cochrane | no estimate extracted | NR | No studies |
Dornase alfa vs placebo or no dornase alfa treatment Relative mean percentage change in FEV1 (% predicted) at 3 months · Adults and children with cystic fibrosis Dornase alfa for cystic fibrosis (2021) · Cochrane | no estimate extracted | 1 study · 320 participants | Moderate |
Dornase alfa daily vs alternate days Mean relative percentage in quality of life score at 3 months · Children with cystic fibrosis Dornase alfa for cystic fibrosis (2021) · Cochrane | MD 0.01 | 43 participants | Low |
Dornase alfa daily vs alternate days Number of pulmonary exacerbations at 3 months · Children with cystic fibrosis Dornase alfa for cystic fibrosis (2021) · Cochrane | no estimate extracted | 43 participants | Low |
Dornase alfa vs hypertonic saline Mean relative percentage in FEV1 (L) at 3 months · Children with cystic fibrosis Dornase alfa for cystic fibrosis (2021) · Cochrane | MD 8 [2, 14] | 431 participants | Low |
Dornase alfa vs hypertonic saline Mean relative percentage in FVC (L) at 3 months · Children with cystic fibrosis Dornase alfa for cystic fibrosis (2021) · Cochrane | MD 0.08 | 431 participants | Low |
Dornase alfa vs hypertonic saline Mean relative percentage in quality of life score at 3 months · Children with cystic fibrosis Dornase alfa for cystic fibrosis (2021) · Cochrane | MD 0.03 | 431 participants | Low |
Dornase alfa vs hypertonic saline Number of pulmonary exacerbations at 3 months · Children with cystic fibrosis Dornase alfa for cystic fibrosis (2021) · Cochrane | no estimate extracted | 431 participants | Low |
Dornase alfa vs mannitol Mean absolute change in FEV1 (L) at 3 months · Children with cystic fibrosis Dornase alfa for cystic fibrosis (2021) · Cochrane | MD 0.02 | 231 participants | Low |
Dornase alfa vs mannitol Mean absolute change in FVC (L) at 3 months · Children with cystic fibrosis Dornase alfa for cystic fibrosis (2021) · Cochrane | no estimate extracted | 231 participants | Low |
Dornase alfa vs mannitol Change in quality of life ‐ CFQ‐R at 3 months · Children with cystic fibrosis Dornase alfa for cystic fibrosis (2021) · Cochrane | MD 10.61 [0.27, 20.95] | 231 participants | Low |
Dornase alfa vs mannitol Number of people experiencing exacerbations ‐ at 3 months · Children with cystic fibrosis Dornase alfa for cystic fibrosis (2021) · Cochrane | RR 1.1 [0.25, 4.84] | 231 participants | Low |
Dornase alfa vs placebo or no dornase alfa treatment Relative mean percentage change in FEV1 (% predicted) at 6 months · Adults and children with cystic fibrosis Dornase alfa for cystic fibrosis (2021) · Cochrane | no estimate extracted | 1 study · 647 participants | High |
Dornase alfa vs dornase alfa and mannitol Mean absolute change in FEV1 (L) at 3 months · Children with cystic fibrosis Dornase alfa for cystic fibrosis (2021) · Cochrane | MD 0.1 | 231 participants | Low |
Dornase alfa vs dornase alfa and mannitol Mean absolute change in FVC (L) at 3 months · Children with cystic fibrosis Dornase alfa for cystic fibrosis (2021) · Cochrane | MD 0.13 | 231 participants | Low |
Dornase alfa vs dornase alfa and mannitol Change in quality of life ‐ CFQ‐R at 3 months · Children with cystic fibrosis Dornase alfa for cystic fibrosis (2021) · Cochrane | MD 10.61 [0.27, 20.95] | 231 participants | Low |
Dornase alfa vs dornase alfa and mannitol Number of people experiencing exacerbations at 3 months · Children with cystic fibrosis Dornase alfa for cystic fibrosis (2021) · Cochrane | RR 0.55 [0.16, 1.92] | 231 participants | Low |
Dornase alfa vs placebo or no dornase alfa treatment Relative mean percentage change in FVC (% predicted) at 3 months · Adults and children with cystic fibrosis Dornase alfa for cystic fibrosis (2021) · Cochrane | no estimate extracted | 1 study · 318 participants | Moderate |
Dornase alfa vs placebo or no dornase alfa treatment Relative mean percentage change in FVC (% predicted) at 6 months · Adults and children with cystic fibrosis Dornase alfa for cystic fibrosis (2021) · Cochrane | MD 3.8 [2.62, 4.98] | 1 study · 647 participants | High |
Dornase alfa vs placebo or no dornase alfa treatment Change in quality of life ‐ CFQ‐R respiratory at 1 month · Adults and children with cystic fibrosis Dornase alfa for cystic fibrosis (2021) · Cochrane | MD 0.84 | 19 participants | Low |
Dornase alfa vs placebo or no dornase alfa treatment Change in quality of life ‐ CFQ‐R respiratory (parent) at 1 month · Adults and children with cystic fibrosis Dornase alfa for cystic fibrosis (2021) · Cochrane | MD 9.78 | 19 participants | Low |
Dornase alfa vs placebo or no dornase alfa treatment Number of people experiencing exacerbations at up to 2 years · Adults and children with cystic fibrosis Dornase alfa for cystic fibrosis (2021) · Cochrane | RR 0.78 [0.62, 0.96] | 3 studies · 1157 participants | Moderate |
Dornase alfa daily vs alternate days Mean relative percentage change in FEV1 (L) at 3 months · Children with cystic fibrosis Dornase alfa for cystic fibrosis (2021) · Cochrane | MD 2 | 43 participants | Low |
Dornase alfa daily vs alternate days Mean relative percentage in FVC (L) at 3 months · Children with cystic fibrosis Dornase alfa for cystic fibrosis (2021) · Cochrane | MD 0.03 | 43 participants | Low |