Cochrane Review Group

Cystic Fibrosis and Genetic Disorders

114 systematic reviews in Cystic Fibrosis and Genetic Disorders ask 2,280 questions. 37.5% have no studies to answer them, 24.1% rest on very low certainty evidence and 13.8% reach moderate or high certainty.

Reviews

114

Questions

2,280

current versions

No studies

37.5%

Moderate or high

13.8%

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.

Search questions in Cystic Fibrosis and Genetic Disorders

Thinnest evidence

All no studies and very low questions

Question	Effect	Evidence	Status / GRADE
Summary of findings ‐ high‐dose azithromycin vs low‐dose azithromycin Adverse events · children with cystic fibrosis Macrolide antibiotics (including azithromycin) for cystic fibrosis (2024) · Cochrane	no estimate extracted	NR	No studies
Summary of findings ‐ high‐dose azithromycin vs low‐dose azithromycin Hospitalisations · children with cystic fibrosis Macrolide antibiotics (including azithromycin) for cystic fibrosis (2024) · Cochrane	no estimate extracted	NR	No studies
Summary of findings ‐ high‐dose azithromycin vs low‐dose azithromycin New acquisition of CF pathogens: PA · children with cystic fibrosis Macrolide antibiotics (including azithromycin) for cystic fibrosis (2024) · Cochrane	no estimate extracted	NR	No studies
Summary of findings ‐ high‐dose azithromycin vs low‐dose azithromycin Need for additional antibiotics · children with cystic fibrosis Macrolide antibiotics (including azithromycin) for cystic fibrosis (2024) · Cochrane	no estimate extracted	NR	No studies
Summary of findings ‐ high‐dose azithromycin vs low‐dose azithromycin QoL · children with cystic fibrosis Macrolide antibiotics (including azithromycin) for cystic fibrosis (2024) · Cochrane	no estimate extracted	NR	No studies
Summary of findings ‐ nebulised azithromycin vs oral azithromycin FEV1 % predicted: mean change from baseline · children with cystic fibrosis Macrolide antibiotics (including azithromycin) for cystic fibrosis (2024) · Cochrane	no estimate extracted	1 study · 45 participants	Very low
Summary of findings ‐ nebulised azithromycin vs oral azithromycin Pulmonary exacerbations: time to next exacerbation · children with cystic fibrosis Macrolide antibiotics (including azithromycin) for cystic fibrosis (2024) · Cochrane	no estimate extracted	NR	No studies
Summary of findings ‐ nebulised azithromycin vs oral azithromycin Adverse events: number of treatment‐related adverse events · children with cystic fibrosis Macrolide antibiotics (including azithromycin) for cystic fibrosis (2024) · Cochrane	no estimate extracted	1 study · 45 participants	Very low
Summary of findings ‐ nebulised azithromycin vs oral azithromycin Hospitalisations · children with cystic fibrosis Macrolide antibiotics (including azithromycin) for cystic fibrosis (2024) · Cochrane	no estimate extracted	NR	No studies
Summary of findings ‐ nebulised azithromycin vs oral azithromycin New acquisition of CF pathogens: PA (mean change in PA count (log10 PA CFU/g sputum)) · children with cystic fibrosis Macrolide antibiotics (including azithromycin) for cystic fibrosis (2024) · Cochrane	no estimate extracted	1 study · 45 participants	Very low
Summary of findings ‐ nebulised azithromycin vs oral azithromycin Need for additional antibiotics · children with cystic fibrosis Macrolide antibiotics (including azithromycin) for cystic fibrosis (2024) · Cochrane	no estimate extracted	NR	No studies
Summary of findings ‐ nebulised azithromycin vs oral azithromycin QoL: change in PEDsQL‐4 scale · children with cystic fibrosis Macrolide antibiotics (including azithromycin) for cystic fibrosis (2024) · Cochrane	no estimate extracted	1 study · 45 participants	Very low

Reviews 114

Digital technology for delivering and monitoring exercise programs for people with cystic fibrosis

Pinto, ACPereira Nunes, Piva, SR, et al. · Cochrane Database of Systematic Reviews · 2023

Cystic Fibrosis and Genetic Disorders

Thinnest evidence

Reviews 114

Digital technology for delivering and monitoring exercise programs for people with cystic fibrosis

Digital technology for early identification of exacerbations in people with cystic fibrosis

Inhaled anti‐pseudomonal antibiotics for long‐term therapy in cystic fibrosis

Hydroxyurea (hydroxycarbamide) for sickle cell disease

Physical activity and exercise training in cystic fibrosis

Appetite stimulants for people with cystic fibrosis

Inhaled antibiotics for pulmonary exacerbations in cystic fibrosis

Interventions for the eradication of meticillin‐resistant Staphylococcus aureus (MRSA) in people with cystic fibrosis

Laser therapy for retinopathy in sickle cell disease

Interventions for reducing inflammation in familial Mediterranean fever

Inhaled nitric oxide for treating pain crises in people with sickle cell disease

Exercise versus airway clearance techniques for people with cystic fibrosis

Statins for Smith‐Lemli‐Opitz syndrome

Short‐acting inhaled bronchodilators for cystic fibrosis

Dornase alfa for cystic fibrosis

Single versus combination intravenous anti‐pseudomonal antibiotic therapy for people with cystic fibrosis

Dietary advice with or without oral nutritional supplements for disease‐related malnutrition in adults

Prophylactic antibiotics for preventing pneumococcal infection in children with sickle cell disease

Recombinant growth hormone therapy for X‐linked hypophosphatemia in children

Timing of dornase alfa inhalation for cystic fibrosis

Interventions for treating leg ulcers in people with sickle cell disease

Recombinant growth hormone therapy for cystic fibrosis in children and young adults

Angiotensin‐converting enzyme (ACE) inhibitors for proteinuria and microalbuminuria in people with sickle cell disease

Autogenic drainage for airway clearance in cystic fibrosis

Enzyme replacement therapy with galsulfase for mucopolysaccharidosis type VI