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

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.

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...

Pancreatic Enzyme Replacement Therapy

Patients with chronic pancreatic insufficiency develop improper digestion due to the lack of digestive pancreatic enzymes. Such patients might complain of diarrhea and multi-nutrient deficiencies. Children with cystic fibrosis are at an increased risk of developing pancreatic insufficiency, while adults can develop chronic pancreatitis due to gallbladder disease or alcohol consumption. Regardle...

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...