CFDB - Cystic Fibrosis DataBase

Cochrane Database of Systematic Reviews - Cochrane Protocol (ongoing review)

Dosing regimens for pancreatic enzyme replacement therapy (PERT) in cystic fibrosis

Study design (if review, criteria of inclusion for studies)

Randomised controlled trials (RCTs), including cross‐over RCTs with a minimum washout period of two weeks. Quasi‐RCTs if baseline characteristics of intervention groups are similar (Higgins 2011b)


Individuals of all ages, with a confirmed diagnosis of CF by genotype or sweat chloride testing, with and without PI.


Regimens pre‐specifying different administration timings (e.g. before, during or after a meal) in any dosage (dose/kg body weight or dose/g ingested fat or any other strategy) or formulation of PERT in people, of any age, with CF.

Outcome measures

Primary outcomes: Fat malabsorption (absolute CFA based on 72‐hour stool collection); Nutritional status (change from baseline); weight in kg, % of predicted weight or z score; height in cm, % of predicted height or z score; BMI, % of predicted BMI or z score; Adverse events

Keywords: Gastrointestinal Diseases; pharmacological_intervention; Pancreas insufficiency; Pancreatic Diseases; Pancreatic Enzyme Replacement Therapy; Malabsorption; Nutrition Disorders; Gastrointestinal Agents;