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Search review questions

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.

Limit to evidence status or GRADE (optional)

2,280 questions in 114 reviews · Cystic Fibrosis and Genetic Disorders

current versions
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.

QuestionEffectEvidenceStatus / GRADE
Autogenic drainage vs intrapulmonary percussive ventilation (200 bpm)
Adverse events · adults with CF
Autogenic drainage for airway clearance in cystic fibrosis (2021) · Cochrane
no estimate extractedNRNo studies
Autogenic drainage vs intrapulmonary percussive ventilation (200 bpm)
Number of admissions to hospital · adults with CF
Autogenic drainage for airway clearance in cystic fibrosis (2021) · Cochrane
no estimate extractedNRNo studies
Autogenic drainage vs intrapulmonary percussive ventilation (200 bpm)
Need for extra treatment · adults with CF
Autogenic drainage for airway clearance in cystic fibrosis (2021) · Cochrane
no estimate extractedNRNo studies
Autogenic drainage vs intrapulmonary percussive ventilation (400 bpm)
FEV1 % predicted (change from baseline) · adults with CF
Autogenic drainage for airway clearance in cystic fibrosis (2021) · Cochrane
no estimate extracted1 study · 4 participantsVery low
Autogenic drainage vs intrapulmonary percussive ventilation (400 bpm)
QoL · adults with CF
Autogenic drainage for airway clearance in cystic fibrosis (2021) · Cochrane
no estimate extractedNRNo studies
Autogenic drainage vs intrapulmonary percussive ventilation (400 bpm)
Participant preference · adults with CF
Autogenic drainage for airway clearance in cystic fibrosis (2021) · Cochrane
no estimate extractedNRNo studies
Autogenic drainage vs conventional physiotherapy
Number of admissions to hospital · adults and children with CF
Autogenic drainage for airway clearance in cystic fibrosis (2021) · Cochrane
no estimate extracted1 study · 36 participantsVery low
Autogenic drainage vs intrapulmonary percussive ventilation (400 bpm)
Exercise tolerance (modified shuttle test) · adults with CF
Autogenic drainage for airway clearance in cystic fibrosis (2021) · Cochrane
no estimate extractedNRNo studies
Autogenic drainage vs intrapulmonary percussive ventilation (400 bpm)
Adverse events · adults with CF
Autogenic drainage for airway clearance in cystic fibrosis (2021) · Cochrane
no estimate extractedNRNo studies
Autogenic drainage vs intrapulmonary percussive ventilation (400 bpm)
Number of admissions to hospital · adults with CF
Autogenic drainage for airway clearance in cystic fibrosis (2021) · Cochrane
no estimate extractedNRNo studies
Autogenic drainage vs intrapulmonary percussive ventilation (400 bpm)
Need for extra treatment · adults with CF
Autogenic drainage for airway clearance in cystic fibrosis (2021) · Cochrane
no estimate extractedNRNo studies
Autogenic drainage vs conventional physiotherapy
Need for extra treatment · adults and children with CF
Autogenic drainage for airway clearance in cystic fibrosis (2021) · Cochrane
no estimate extracted1 study · 36 participantsVery low
Autogenic drainage vs spontaneous cough
FEV1 % predicted · adults and children with CF
Autogenic drainage for airway clearance in cystic fibrosis (2021) · Cochrane
no estimate extracted1 study · 14 participantsVery low
Autogenic drainage vs spontaneous cough
QoL · adults and children with CF
Autogenic drainage for airway clearance in cystic fibrosis (2021) · Cochrane
no estimate extractedNRNo studies
Intervention NR
12MWT: change from baseline in m walked · children and adults with mucopolysaccharidosis type VI
Enzyme replacement therapy with galsulfase for mucopolysaccharidosis type VI (2021) · Cochrane
no estimate extracted1 study · 38 participantsVery low
Intervention NR
3MSC: change in 3MSC rates (stairs/min) from baseline · children and adults with mucopolysaccharidosis type VI
Enzyme replacement therapy with galsulfase for mucopolysaccharidosis type VI (2021) · Cochrane
no estimate extracted1 study · 38 participantsVery low
Intervention NR
6MWT: change from baseline in m walked · children and adults with mucopolysaccharidosis type VI
Enzyme replacement therapy with galsulfase for mucopolysaccharidosis type VI (2021) · Cochrane
no estimate extracted1 study · 38 participantsVery low
Intervention NR
Respiratory tests: absolute change from baseline in FVC (L) · children and adults with mucopolysaccharidosis type VI
Enzyme replacement therapy with galsulfase for mucopolysaccharidosis type VI (2021) · Cochrane
no estimate extracted1 study · 38 participantsVery low
Intervention NR
Cardiac function  · children and adults with mucopolysaccharidosis type VI
Enzyme replacement therapy with galsulfase for mucopolysaccharidosis type VI (2021) · Cochrane
no estimate extractedNRNo studies
Intervention NR
GAG level: change in urinary GAG level from baseline ųg/mg creatinine · children and adults with mucopolysaccharidosis type VI
Enzyme replacement therapy with galsulfase for mucopolysaccharidosis type VI (2021) · Cochrane
no estimate extracted1 study · 38 participantsLow
Intervention NR
AEs: drug‐related AEs · children and adults with mucopolysaccharidosis type VI
Enzyme replacement therapy with galsulfase for mucopolysaccharidosis type VI (2021) · Cochrane
RR 1.93 [0.89, 4.17]1 study · 39 participantsVery low
Cisapride vs placebo
Radiological diagnosis of DIOS (physician‐measured radiological scores) · people with cystic fibrosis
Interventions for preventing distal intestinal obstruction syndrome (DIOS) in cystic fibrosis (2021) · Cochrane
no estimate extracted1 study · 17 participantsVery low
Cisapride vs placebo
Adverse effects (participant interviews) · people with cystic fibrosis
Interventions for preventing distal intestinal obstruction syndrome (DIOS) in cystic fibrosis (2021) · Cochrane
no estimate extracted1 study · 17 participantsVery low
Cisapride vs placebo
Total gastrointestinal symptom scores (participant‐reported symptom scores from 20 to 100) · people with cystic fibrosis
Interventions for preventing distal intestinal obstruction syndrome (DIOS) in cystic fibrosis (2021) · Cochrane
no estimate extracted1 study · 17 participantsVery low
Cisapride vs placebo
Hospitalisation for any cause · people with cystic fibrosis
Interventions for preventing distal intestinal obstruction syndrome (DIOS) in cystic fibrosis (2021) · Cochrane
no estimate extractedNRNo studies
Cisapride vs placebo
Hospitalisation for DIOS · people with cystic fibrosis
Interventions for preventing distal intestinal obstruction syndrome (DIOS) in cystic fibrosis (2021) · Cochrane
no estimate extractedNRNo studies
Cisapride vs placebo
Quality of life · people with cystic fibrosis
Interventions for preventing distal intestinal obstruction syndrome (DIOS) in cystic fibrosis (2021) · Cochrane
no estimate extractedNRNo studies
Cisapride vs placebo
Tolerability · people with cystic fibrosis
Interventions for preventing distal intestinal obstruction syndrome (DIOS) in cystic fibrosis (2021) · Cochrane
no estimate extractedNRNo studies
Summary of findings ‐ high‐dose pancreatic enzymes vs low‐dose pancreatic enzymes
Time taken from start of treatment until the resolution of DIOS · children and adults with cystic fibrosis
Interventions for treating distal intestinal obstruction syndrome (DIOS) in cystic fibrosis (2021) · Cochrane
no estimate extractedNRNo studies
Summary of findings ‐ high‐dose pancreatic enzymes vs low‐dose pancreatic enzymes
Treatment failure rate · children and adults with cystic fibrosis
Interventions for treating distal intestinal obstruction syndrome (DIOS) in cystic fibrosis (2021) · Cochrane
no estimate extractedNRNo studies
Summary of findings ‐ high‐dose pancreatic enzymes vs low‐dose pancreatic enzymes
Adherence · children and adults with cystic fibrosis
Interventions for treating distal intestinal obstruction syndrome (DIOS) in cystic fibrosis (2021) · Cochrane
no estimate extractedNRNo studies
Summary of findings ‐ high‐dose pancreatic enzymes vs low‐dose pancreatic enzymes
Episodes of acute DIOS · children and adults with cystic fibrosis
Interventions for treating distal intestinal obstruction syndrome (DIOS) in cystic fibrosis (2021) · Cochrane
no estimate extracted1 study · 20 participantsVery low
Summary of findings ‐ high‐dose pancreatic enzymes vs low‐dose pancreatic enzymes
Adverse effects: abdominal mass · children and adults with cystic fibrosis
Interventions for treating distal intestinal obstruction syndrome (DIOS) in cystic fibrosis (2021) · Cochrane
no estimate extracted1 study · 20 participantsVery low
Summary of findings ‐ high‐dose pancreatic enzymes vs low‐dose pancreatic enzymes
Adverse effects: abdominal pain · children and adults with cystic fibrosis
Interventions for treating distal intestinal obstruction syndrome (DIOS) in cystic fibrosis (2021) · Cochrane
no estimate extracted1 study · 20 participantsVery low
Summary of findings ‐ AZLI vs placebo
Relative change in FEV1% predicted · adults and children with CF
Antibiotic therapy for chronic infection with Burkholderia cepacia complex in people with cystic fibrosis (2021) · Cochrane
no estimate extracted1 study · 99 participantsModerate
Summary of findings ‐ AZLI vs placebo
Days to next exacerbation · adults and children with CF
Antibiotic therapy for chronic infection with Burkholderia cepacia complex in people with cystic fibrosis (2021) · Cochrane
no estimate extracted1 study · 100 participantsModerate
Summary of findings ‐ AZLI vs placebo
Participants hospitalised for respiratory exacerbation · adults and children with CF
Antibiotic therapy for chronic infection with Burkholderia cepacia complex in people with cystic fibrosis (2021) · Cochrane
RR 0.88 [0.53, 1.45]1 study · 100 participantsModerate
Summary of findings ‐ AZLI vs placebo
Any AEs · adults and children with CF
Antibiotic therapy for chronic infection with Burkholderia cepacia complex in people with cystic fibrosis (2021) · Cochrane
RR 1.08 [0.98, 1.19]1 study · 100 participantsModerate
Summary of findings ‐ AZLI vs placebo
Mortality · adults and children with CF
Antibiotic therapy for chronic infection with Burkholderia cepacia complex in people with cystic fibrosis (2021) · Cochrane
RR 5.41 [0.27, 109.87]1 study · 100 participantsModerate
Intervention NR
Number of joint bleeding episodes per year (AJBR) · children or adults with hemophilia A or B
Clotting factor concentrates for preventing bleeding and bleeding‐related complications in previously… (2021) · Cochrane
no estimate extracted3 studies · 219 participantsLow
Intervention NR
Number of total bleeds per year (ABR) · children or adults with hemophilia A or B
Clotting factor concentrates for preventing bleeding and bleeding‐related complications in previously… (2021) · Cochrane
no estimate extracted7 studies · 310 participantsLow
Intervention NR
Treatment‐related adverse events · children or adults with hemophilia A or B
Clotting factor concentrates for preventing bleeding and bleeding‐related complications in previously… (2021) · Cochrane
no estimate extracted3 studies · 223 participantsVery low
Prophylaxis with standard therapeutic factor concentrate vs pegylated liposome FVIII formulation
AJBR · children or adults with hemophilia A
Clotting factor concentrates for preventing bleeding and bleeding‐related complications in previously… (2021) · Cochrane
no estimate extracted1 study · 143 participantsLow
Prophylaxis with standard therapeutic factor concentrate vs pegylated liposome FVIII formulation
ABR · children or adults with hemophilia A
Clotting factor concentrates for preventing bleeding and bleeding‐related complications in previously… (2021) · Cochrane
no estimate extracted1 study · 143 participantsLow
Prophylaxis with standard therapeutic factor concentrate vs pegylated liposome FVIII formulation
Any reported adverse effects · children or adults with hemophilia A
Clotting factor concentrates for preventing bleeding and bleeding‐related complications in previously… (2021) · Cochrane
no estimate extracted1 study · 143 participantsLow
Prophylaxis regimen vs on‐demand treatment
Number of joint bleeding episodes or joint bleeding frequency · children and adults with haemophilia A or B
Clotting factor concentrates for preventing bleeding and bleeding‐related complications in previously… (2021) · Cochrane
no estimate extracted2 studies · 164 participantsLow
Prophylaxis regimen vs on‐demand treatment
Number of total bleeds per year or bleeding frequency · children and adults with haemophilia A or B
Clotting factor concentrates for preventing bleeding and bleeding‐related complications in previously… (2021) · Cochrane
no estimate extracted2 studies · 164 participantsLow
Prophylaxis regimen vs on‐demand treatment
Any reported adverse events · children and adults with haemophilia A or B
Clotting factor concentrates for preventing bleeding and bleeding‐related complications in previously… (2021) · Cochrane
RR 1.71 [1.24, 2.37]2 studies · 131 participantsModerate
Summary of findings ‐ Prophylactic vs 'as required' anti‐staphylococcal antibiotics
FEV1 (% predicted (Knudson 1983)) · children with cystic fibrosis
Prophylactic anti‐staphylococcal antibiotics for cystic fibrosis (2020) · Cochrane
no estimate extracted1 study · 119 participantsLow
Summary of findings ‐ Prophylactic vs 'as required' anti‐staphylococcal antibiotics
Number of people with one or more isolates of S aureus (sensitive strains) · children with cystic fibrosis
Prophylactic anti‐staphylococcal antibiotics for cystic fibrosis (2020) · Cochrane
OR 0.21 [0.13, 0.35]3 studies · 315 participantsLow