Bile Salt Kinetics in Cystic Fibrosis: Influence of Pancreatic Enzyme Replacement

Pancreatic enzyme supplementation in cystic fibrosis.

Pancreatic enzyme supplements in cystic fibrosis.

Approximately 95% of patients in Northern Europe are pancreatic insufficient due to an inadequacy of their own pancreatic enzyme secretions (Littlewood et al, 2006). These patients need to take pancreatic enzyme replacement therapy (PERT) in order to prevent the symptoms of fat malabsorption. These symptoms include frequent pale, oily and offensive stools, abdominal pain, poor growth and defici...

Pancreatic enzyme replacement therapy in cystic fibrosis: dose, variability and coefficient of fat absorption.

OBJECTIVES Pancreatic enzyme replacement therapy (PERT) remains a backbone in the nutritional treatment of cystic fibrosis. Currently, there is a lack of an evidence-based tool that allows dose adjustment. To date, no studies have found an association between PERT dose and fat absorption. Therefore, the aim of the study was to assess the influence of both the PERT dose and the variability in th...

Symptomatic increase in intracranial pressure following pancreatic enzyme replacement therapy for cystic fibrosis.

A newly diagnosed 5-month-old infant with cystic fibrosis (CF) developed signs and symptoms of increased intracranial pressure (ICP) within days of starting pancreatic enzyme replacement therapy. Symptoms promptly resolved on two occasions after stopping enzyme replacement. At 10 months of age, enzyme replacement was well tolerated.

Effect of cimetidine on enzyme inactivation, bile acid precipitation, and lipid solubilisation in pancreatic steatorrhoea due to cystic fibrosis.

In pancreatic steatorrhoea, both pH-dependent bile acid precipitation and enzyme inactivation may limit the efficacy of pancreatic enzyme supplements and both may be preventable by addition of cimetidine. To separate these effects we compared postprandial jejunal aspirate from eight adults with steatorrhoea due to cystic fibrosis on three randomised treatment regimens (pancreatin, cimetidine, a...