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

Interventions for preventing distal intestinal obstruction syndrome (DIOS) in cystic fibrosis

Carroll, W, Green, J, et al. · Cochrane Database of Systematic Reviews · 2021

Cystic Fibrosis and Genetic Disorders

Thinnest evidence

Reviews 114

Interventions for preventing distal intestinal obstruction syndrome (DIOS) in cystic fibrosis

Interventions for treating distal intestinal obstruction syndrome (DIOS) in cystic fibrosis

Antibiotic therapy for chronic infection with Burkholderia cepacia complex in people with cystic fibrosis

Clotting factor concentrates for preventing bleeding and bleeding‐related complications in previously treated individuals with haemophilia A or B

Prophylactic anti‐staphylococcal antibiotics for cystic fibrosis

Omega‐3 fatty acid supplementation for cystic fibrosis

Blood transfusion for preventing primary and secondary stroke in people with sickle cell disease

Preoperative blood transfusions for sickle cell disease

Treatments for priapism in boys and men with sickle cell disease

Phytomedicines (medicines derived from plants) for sickle cell disease

Drug treatments for managing cystic fibrosis‐related diabetes

Respiratory muscle training for cystic fibrosis

Oscillating devices for airway clearance in people with cystic fibrosis

Combination antimicrobial susceptibility testing for acute exacerbations in chronic infection of Pseudomonas aeruginosa in cystic fibrosis

Antibiotic adjuvant therapy for pulmonary infection in cystic fibrosis

Pancreatic enzyme replacement therapy for people with cystic fibrosis

Pine bark (Pinus spp.) extract for treating chronic disorders

Vitamin K supplementation for cystic fibrosis

Inhaled mannitol for cystic fibrosis

Timing of hypertonic saline inhalation for cystic fibrosis

Vitamin E supplementation in people with cystic fibrosis

Standard versus biofilm antimicrobial susceptibility testing to guide antibiotic therapy in cystic fibrosis

Psychological interventions for people with hemophilia

Vitamin D supplementation for sickle cell disease

Growth hormone therapy for people with thalassaemia