Cystic fibrosis pancreatic enzyme supplementation

Exocrine pancreatic insufficiency is most commonly caused by cystic fibrosis, chronic pancreatitis, or pancreatic resection. When secretion of pancreatic enzymes falls below 10% of normal, fat and protein digestion is impaired and can lead to steatorrhea, azotorrhea, vitamin malabsorption, and weight loss. Pancreatic enzyme supplements, which contain a mixture of amylase, lipase, and proteases, are the mainstay of treatment for pancreatic enzyme insufficiency. Two major types of preparations in use are pancreatin and pancrelipase. Pancreatin is an alcohol-derived extract of hog pancreas with relatively low concentrations of lipase and proteolytic enzymes, whereas pancrelipase is an enriched preparation. On a per-weight basis, pancrelipase has approximately 12 times the lipolytic activity and more than 4 times the proteolytic activity of pancreatin. Consequently, pancreatin is no longer in common clinical use. Only pancrelipase is discussed here. 

Pancreatic enzyme supplements are well tolerated. The capsules should be swallowed, not chewed, because pancreatic enzymes may cause oropharyngeal mucositis. Excessive doses may cause diarrhea and abdominal pain. The high purine content of pancreas extracts may lead to hyperuricosuria and renal stones. Several cases of colonic strictures were reported in patients with cystic fibrosis who received high doses of pancrelipase with high lipase activity. These high-dose formulations have since been removed from the market. 

C. J. Taylor and J. A. Dodge. High-strength pancreatic enzyme supplements and large-bowel stricture in cystic fibrosis. Lancet 5 110(1994). 

Colonic toxicity due to pancreatic enzyme supplements in children with cystic fibrosis has been reviewed (3). Since it was first reported in 1994, an increasing number of cases of fibrosing colonopathy due to pancreatic enzymes have been reported in children with cystic fibrosis in the UK, USA, and continental Europe. The disorder is not affected by age or sex, and the age at diagnosis is 9 months to 13 years. 

Amarri S, Harding M, Coward WA, Evans TJ, Weaver LT. C-mixed triglyceride breath test and pancreatic enzyme supplementation in children with cystic fibrosis. Arch Dis Childhood 1997 76 349-51. 

FitzSimmons SC, Burkhart GA, Borowitz D, et al. High-dose pancreatic-enzyme supplements and fibrosing colonopathy in children with cystic fibrosis. N Engl J Med 1997 336 1283-1289. 

Pancreatic enzyme replacement or supplement when enzymes are absent or deficient, such as with chronic pancreatitis, cystic fibrosis, or ductal obstruction from cancer of the pancreas or common bile duct to reduce malabsorption treatment of steatorrhea associated with bowel resection or postgastrectomy syndrome PO 1-3 capsules ortablets before or with meals or snacks. May increase to 8 tablets/dose. 

Nutrient malabsorption also occurs in the genetic disease cystic fibrosis. This disease arises from a mutation in the chloride ion transporter, and results in pancreatic insufficiency as well a.s respiratory difficulties. Treatment of the malabsorption symptoms of cystic fibrosis involves supplementation with pancreatic enzymes and synthetic water-soluble versions of the fat-soluble vitamins. The labored breathing and respiratory infections due to the disease cannot be treated by dietary intervention. 

V. Hubbard, G. Dunn, and L. Lester. Effectiveness of cimetidine as an adjunct to supplemental pancreatic enzymes in patients with cystic fibrosis. Am. J. Clin. Nutr. 33 2281 (1980). 

Drug formulations Impaired digestion in cystic fibrosis affects about 90% of patients. As soon as pancreatic insufficiency is identified, enzyme supplementation is prescribed, even for breast fed infants. In a prospective, randomized study 40 infants and toddlers were treated with Creon for children, a formulation that contains smaller granules and is administered with a dosing spoon (5000 lipase units per scoop) and Creon 10 000 for 2 weeks each in a crossover design [113 ]. The former was superior in terms of parents preference, but equally effective with regard to fat absorption. Three patients who took Creon for children had treatment-related adverse events abdominal pain, constipation, vomiting, with one withdrawal) compared with one who took Creon 10 000 (severe diaper dermatitis/nappy rash). 

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