What do systematic reviews say?

Summary of findings: ECM vs TPE

The review found no eligible studies for this question.

• Pancreatic enzyme replacement therapy for people with cystic fibrosis 2020 · Cochrane Headline outcomes only
  no estimate extracted
  Studies not reported
  No studies found

Summary of findings: ECM vs other enteric‐coated preparations

The review found no eligible studies for this question.

• Pancreatic enzyme replacement therapy for people with cystic fibrosis 2020 · Cochrane Headline outcomes only
  no estimate extracted
  Studies not reported
  No studies found

Summary of findings: low‐dose vs high‐dose PERT

The review found no eligible studies for this question.

• Pancreatic enzyme replacement therapy for people with cystic fibrosis 2020 · Cochrane Headline outcomes only
  no estimate extracted
  Studies not reported
  No studies found

Summary of findings: liprotamase vs porcine PERT

Summary of findings: liprotamase showed no clear difference in pulmonary exacerbations: number of exacerbations compared with porcine PERT (low certainty).

• Pancreatic enzyme replacement therapy for people with cystic fibrosis 2020 · Cochrane Headline outcomes only
  OR 0.56 [0.13, 2.45]
  1 study · 128 participants
  Low

Summary of findings: ECM vs NECT plus cimetidine

The review found no eligible studies for this question.

• Pancreatic enzyme replacement therapy for people with cystic fibrosis 2020 · Cochrane Headline outcomes only
  no estimate extracted
  Studies not reported
  No studies found

Probiotics vs placebo

• Probiotics for people with cystic fibrosis 2020 · Cochrane Headline outcomes only
  no estimate extracted
  4 studies · 225 participants
  Low

Summary of findings: oral vitamin E supplementation vs placebo or no treatment

The review found no eligible studies for this question.

• Antioxidant supplementation for lung disease in cystic fibrosis 2019 · Cochrane Headline outcomes only
  no estimate extracted
  Studies not reported
  No studies found

Summary of findings: oral β‐carotene vs placebo

The review found no eligible studies for this question.

• Antioxidant supplementation for lung disease in cystic fibrosis 2019 · Cochrane Headline outcomes only
  no estimate extracted
  Studies not reported
  No studies found

Summary of findings: oral antioxidant combination vs control

The review found no eligible studies for this question.

• Antioxidant supplementation for lung disease in cystic fibrosis 2019 · Cochrane Headline outcomes only
  no estimate extracted
  Studies not reported
  No studies found

Summary of findings: oral antioxidant combination vs control

• Antioxidant supplementation for lung disease in cystic fibrosis 2019 · Cochrane Headline outcomes only
  no estimate extracted
  1 study · 69 participants
  Low

Summary of findings: inhaled antioxidants vs placebo

The review found no eligible studies for this question.

• Antioxidant supplementation for lung disease in cystic fibrosis 2019 · Cochrane Headline outcomes only
  no estimate extracted
  Studies not reported
  No studies found

Summary of findings: oral antioxidants (NAC/GSH) vs placebo

The review found no eligible studies for this question.

• Antioxidant supplementation for lung disease in cystic fibrosis 2019 · Cochrane Headline outcomes only
  no estimate extracted
  Studies not reported
  No studies found

Summary of findings ‐ ivacaftor vs placebo

Summary of findings ‐ ivacaftor probably leads to lower time to first pulmonary exacerbation compared with placebo (moderate certainty).

• Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis 2019 · Cochrane Headline outcomes only
  HR 0.46 [0.29, 0.73]
  1 study · 161 participants
  Moderate

Summary of findings ‐ ivacaftor vs placebo

Summary of findings ‐ ivacaftor showed no clear difference in time to first pulmonary exacerbation compared with placebo (moderate certainty).

• Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis 2019 · Cochrane Headline outcomes only
  HR 0.93 [0.42, 2.08]
  1 study · 69 participants
  Moderate

Summary of findings ‐ ivacaftor vs placebo

The review found no eligible studies for this question.

• Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis 2019 · Cochrane Headline outcomes only
  no estimate extracted
  Studies not reported
  No studies found

Summary of findings: cycled vs culture‐based antibiotic therapy

• Treatments for preventing recurrence of infection with Pseudomonas aeruginosa in people with cystic fibrosis 2019 · Cochrane Headline outcomes only
  no estimate extracted
  1 study · 253 participants
  Moderate

Intervention not reported

• Standard (head‐down tilt) versus modified (without head‐down tilt) postural drainage in infants and young children… 2018 · Cochrane Headline outcomes only
  no estimate extracted
  1 study · 20 participants
  Moderate

Intervention not reported

The intervention showed no clear difference in pulmonary function test (FVC) % predicted (moderate certainty).

• Standard (head‐down tilt) versus modified (without head‐down tilt) postural drainage in infants and young children… 2018 · Cochrane Headline outcomes only
  MD 16
  1 study · 14 participants
  Moderate

Beta‐2 agonists vs placebo

The review found no eligible studies for this question.

• Long‐acting inhaled bronchodilators for cystic fibrosis 2017 · Cochrane Headline outcomes only
  no estimate extracted
  Studies not reported
  No studies found

LAMA vs placebo

LAMAs showed no clear difference in pulmonary exacerbations: participants reporting ≥1 pulmonary… compared with placebo (moderate certainty).

• Long‐acting inhaled bronchodilators for cystic fibrosis 2017 · Cochrane Headline outcomes only
  RR 0.6 [0.32, 1.12]
  2 studies · 805 participants
  Moderate