Cochrane · systematic review · CD008227

Pancreatic enzyme replacement therapy for people with cystic fibrosis

Somaraju, URR, Solis-Moya, A · Cochrane Database of Systematic Reviews · 2020 · Cochrane Library

SoF tableOnly the outcomes this review put in its Summary of Findings table are listed (the Cochrane Handbook caps them at about seven per comparison). Its other analyses are not shown here.
Questions
56
current version
GRADE rated
27
No studies
29
Included studies
NR
No studies 29 · 51.8%Ungraded 0 · 0%Very low 16 · 28.6%Low 9 · 16.1%Moderate 2 · 3.6%High 0 · 0%

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.

Questions and results

Summary of findings: ECM compared with NECT plus cimetidine

Population: adults with cystic fibrosis
7 questions
OutcomeEffectEvidenceStatus / GRADE
Change in weight (kg)
Summary of findings: ECM vs NECT plus cimetidine
MD 0.41 study · 12 participantsVery low
Downgraded: risk of bias, imprecision
Change in height
Summary of findings: ECM vs NECT plus cimetidine
no estimate extractedNRNo studies
Change in BMI
Summary of findings: ECM vs NECT plus cimetidine
no estimate extractedNRNo studies
Frequency in bowel symptoms: abdominal pain (% of days affected)
Summary of findings: ECM vs NECT plus cimetidine
no estimate extracted1 study · 12 participantsVery low
Downgraded: risk of bias, imprecision
CFA: change in FFE (g/day)
Summary of findings: ECM vs NECT plus cimetidine
no estimate extracted1 study · 12 participantsLow
Downgraded: risk of bias, imprecision
Adverse events
Summary of findings: ECM vs NECT plus cimetidine
no estimate extractedNRNo studies
Pulmonary exacerbations
Summary of findings: ECM vs NECT plus cimetidine
no estimate extractedNRNo studies

Summary of findings: ECM compared with ECT

Population: children and adults with cystic fibrosis
7 questions
OutcomeEffectEvidenceStatus / GRADE
Change in weight (kg)
Summary of findings: ECM vs ECT
MD 0.322 studies · 41 participantsVery low
Downgraded: risk of bias, imprecision
Change in height
Summary of findings: ECM vs ECT
no estimate extractedNRNo studies
Change in BMI
Summary of findings: ECM vs ECT
no estimate extractedNRNo studies
Frequency of bowel symptoms: abdominal pain (% of days affected)
Summary of findings: ECM vs ECT
no estimate extracted2 studies · 41 participantsVery low
Downgraded: risk of bias, imprecision
CFA: change in FFE (g/day)
Summary of findings: ECM vs ECT
no estimate extracted2 studies · 41 participantsLow
Downgraded: risk of bias, imprecision
Adverse events
Summary of findings: ECM vs ECT
no estimate extractedNRNo studies
Pulmonary exacerbations
Summary of findings: ECM vs ECT
no estimate extractedNRNo studies

Summary of findings: ECM compared with ECMM

Population: children with cystic fibrosis and proven pancreatic insufficiency
7 questions
OutcomeEffectEvidenceStatus / GRADE
Change in weight
Summary of findings: ECM vs ECMM
no estimate extractedNRNo studies
Change in height
Summary of findings: ECM vs ECMM
no estimate extractedNRNo studies
Change in BMI
Summary of findings: ECM vs ECMM
no estimate extractedNRNo studies
Frequency of bowel symptoms
Summary of findings: ECM vs ECMM
no estimate extracted1 study · 54 participantsVery low
Downgraded: risk of bias, imprecision, indirectness
CFA: change in FFE (g/day)
Summary of findings: ECM vs ECMM
no estimate extracted1 study · 22 participantsVery low
Downgraded: risk of bias, imprecision, indirectness
Adverse events
Summary of findings: ECM vs ECMM
no estimate extractedNRNo studies
Pulmonary exacerbations
Summary of findings: ECM vs ECMM
no estimate extractedNRNo studies

Summary of findings: ECM (Creon®) compared with a different ECM

Population: children and adults with cystic fibrosis
7 questions
OutcomeEffectEvidenceStatus / GRADE
Change in weight (kg)
Summary of findings: ECM (Creon®) vs a different ECM
MD 01 study · 83 participantsModerate
Downgraded: risk of bias, imprecision
Change in height
Summary of findings: ECM (Creon®) vs a different ECM
no estimate extractedNRNo studies
Change in BMI
Summary of findings: ECM (Creon®) vs a different ECM
no estimate extractedNRNo studies
Frequency of bowel symptoms: proportion of days with abdominal pain
Summary of findings: ECM (Creon®) vs a different ECM
MD 01 study · 83 participantsLow
Downgraded: risk of bias, imprecision
CFA: CFA (%)
Summary of findings: ECM (Creon®) vs a different ECM
MD 1.352 studies · 110 participantsModerate
Downgraded: risk of bias, imprecision
Adverse events
Summary of findings: ECM (Creon®) vs a different ECM
no estimate extracted1 study · 83 participantsLow
Downgraded: risk of bias, imprecision
Pulmonary exacerbations
Summary of findings: ECM (Creon®) vs a different ECM
no estimate extractedNRNo studies

Summary of findings: ECM compared with TPE

Population: children with cystic fibrosis
7 questions
OutcomeEffectEvidenceStatus / GRADE
Change in weight (kg)
Summary of findings: ECM vs TPE
no estimate extracted1 study · 17 participantsVery low
Downgraded: risk of bias, imprecision, indirectness
Change in height
Summary of findings: ECM vs TPE
no estimate extractedNRNo studies
Change in BMI
Summary of findings: ECM vs TPE
no estimate extractedNRNo studies
Frequency of bowel symptoms
Summary of findings: ECM vs TPE
no estimate extractedNRNo studies
CFA: change in FFE (g/day)
Summary of findings: ECM vs TPE
no estimate extracted1 study · 17 participantsVery low
Downgraded: risk of bias, imprecision, indirectness
Adverse events
Summary of findings: ECM vs TPE
no estimate extractedNRNo studies
Pulmonary exacerbations
Summary of findings: ECM vs TPE
no estimate extractedNRNo studies

Summary of findings: ECM compared with other enteric‐coated preparations

Population: children with cystic fibrosis
7 questions
OutcomeEffectEvidenceStatus / GRADE
Change in weight (kg)
Summary of findings: ECM vs other enteric‐coated preparations
no estimate extracted2 studies · 56 participantsVery low
Downgraded: risk of bias, imprecision, indirectness
Change in height
Summary of findings: ECM vs other enteric‐coated preparations
no estimate extracted1 study · 45 participantsVery low
Downgraded: risk of bias, imprecision, indirectness
Change in BMI
Summary of findings: ECM vs other enteric‐coated preparations
no estimate extractedNRNo studies
Frequency of bowel symptoms: stool frequency
Summary of findings: ECM vs other enteric‐coated preparations
no estimate extracted2 studies · 56 participantsVery low
Downgraded: risk of bias, imprecision, indirectness
CFA: fat absorption
Summary of findings: ECM vs other enteric‐coated preparations
no estimate extracted2 studies · 56 participantsVery low
Downgraded: risk of bias, imprecision, indirectness
Adverse events
Summary of findings: ECM vs other enteric‐coated preparations
no estimate extractedNRNo studies
Pulmonary exacerbations
Summary of findings: ECM vs other enteric‐coated preparations
no estimate extractedNRNo studies

Summary of findings: low‐dose compared with high‐dose PERT

Population: children and adults with cystic fibrosis
7 questions
OutcomeEffectEvidenceStatus / GRADE
Change in weight (kg)
Summary of findings: low‐dose vs high‐dose PERT
no estimate extracted1 study · 17 participantsVery low
Downgraded: risk of bias, imprecision, indirectness
Change in height
Summary of findings: low‐dose vs high‐dose PERT
no estimate extractedNRNo studies
Change in BMI
Summary of findings: low‐dose vs high‐dose PERT
no estimate extractedNRNo studies
Frequency of bowel symptoms: stool frequency
Summary of findings: low‐dose vs high‐dose PERT
no estimate extracted1 study · 17 participantsVery low
Downgraded: risk of bias, imprecision, indirectness
CFA: FFE (g/day)
Summary of findings: low‐dose vs high‐dose PERT
no estimate extracted1 study · 17 participantsVery low
Downgraded: risk of bias, imprecision, indirectness
Adverse events
Summary of findings: low‐dose vs high‐dose PERT
no estimate extracted2 studies · 146 participantsVery low
Downgraded: risk of bias, imprecision, indirectness
Pulmonary exacerbations
Summary of findings: low‐dose vs high‐dose PERT
no estimate extractedNRNo studies

Summary of findings: liprotamase compared with porcine PERT

Population: children aged 7 years or over and adults with cystic fibrosis
7 questions
OutcomeEffectEvidenceStatus / GRADE
Change in weight (kg)
Summary of findings: liprotamase vs porcine PERT
no estimate extracted1 study · 128 participantsLow
Downgraded: risk of bias
Change in height
Summary of findings: liprotamase vs porcine PERT
no estimate extractedNRNo studies
Change in BMI
Summary of findings: liprotamase vs porcine PERT
no estimate extractedNRNo studies
Frequency of bowel symptoms: abdominal pain
Summary of findings: liprotamase vs porcine PERT
no estimate extracted1 study · 128 participantsLow
Downgraded: risk of bias
CFA: change from baseline (%)
Summary of findings: liprotamase vs porcine PERT
no estimate extracted1 study · 128 participantsLow
Downgraded: risk of bias
Adverse events
Summary of findings: liprotamase vs porcine PERT
no estimate extracted1 study · 128 participantsLow
Downgraded: risk of bias
Pulmonary exacerbations: number of exacerbations
Summary of findings: liprotamase vs porcine PERT
OR 0.56 [0.13, 2.45]1 study · 128 participantsLow
Downgraded: risk of bias