CFDB - Cystic Fibrosis DataBase

primary studies published RCT

Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation.

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

double blinded randomised crossover trial.

Participants

14 adolescents (13.1 +/- 2.7 years) with pancreatic insufficient CF and 7 healthy age matched controls.

Interventions

Subjects consumed a high fat pancake, with either PERT (50,000IU lipase) or placebo.

Outcome measures

Post prandial hyperglycaemia measured as peak glucose and area under the curve for blood glucose at 240 minutes.

Main results

CF subjects had postprandial hyperglycaemia compared to controls (area under the curve, P < 0.0001). PERT reduced postprandial hyperglycaemia (P = 0.0002), slowed gastric emptying (P = 0.003), and normalised GLP-1 and GIP secretion (P <0.001 for each) when compared to placebo, without affecting insulin.

Authors' conclusions

In young people with pancreatic insufficient CF, PERT markedly attenuates postprandial hyperglycaemia by slowing gastric emptying and augmenting incretin hormone secretion.

Keywords: Adult; Gastrointestinal Diseases; Hypoglycemic Agents; Insulin; pharmacological_intervention; Pancreas insufficiency; Pancreatic Diseases; Pancreatic Enzyme Replacement Therapy; Supplementation; Malabsorption; Nutrition Disorders; Capsules; Creon; Gastrointestinal Agents;