Cystic fibrosis digestive enzymes

These drug are prescribed as replacement therapy for those with pancreatic enzyme insufficiency. Conditions or diseases that may cause a decrease in or absence of pancreatic digestive enzymes include cystic fibrosis, chronic pancreatitis, cancer of the pancreas,... 

The pancreas, in which the mucus blocks its ducts in 85 per cent of cystic fibrosis patients, causing pancreatic insufficiency. This is chiefly characterized by secretion of greatly reduced levels of digestive enzymes into the small intestine. 

Pancreatin is a pancreatic extract usually obtained from the pancrease of slaughterhouse animals. It contains a mixture of enzymes, principally amylase, protease and lipase, and, thus, exhibits a broad digestive capability. It is administered orally mainly for the treatment of pancreatic insufficiency caused by cystic fibrosis or pancreatitis. As it is sensitive to stomach acid, it must be administered in high doses or, more usually, as enteric-coated granules or capsules that may be taken directly or sprinkled upon the food prior to its ingestion. Individual digestive activities, such as papain, pepsin or bromelains (proteases), or a-amylase are sometimes used in place of pancreatin. 

The use of enzymes as digestive aids is only applied under specific medical circumstances. Some medical conditions (e.g. cystic fibrosis) can result in compromised digestive function due to insufficient production/secretion of endogenous digestive enzymes. Digestive enzyme preparations are often formulated in powder (particularly tablet) form, and are recommended to be taken orally immediately prior to or during meals. As the product never enters the blood... 

Retention of viscous purulent secretions, which contain high concentrations of extracellular DNA—released by degenerating leukocytes that accumulate in response to infection [24]—in the airways contributes both to reduced pulmonary function and to exacerbations of infection [24,25], Digestion of DNA polymers in purulent secretion with DNAse (dornase-a or Pulmozyme) has been shown to reduce sputum viscosity in cystic fibrosis patients. The availability of recombinant DNAse has allowed its use in an aerosol formulation to deliver the enzyme into the deep lung alveoli of CF patients. The purihed glycoprotein contains 260 amino acids with an approximate molecular weight of 37,000 daltons [26], The primary amino-acid sequence is identical to that of the... 

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. 

Pancrelipase Replacement enzymes from animal pancreatic extracts Improves digestion of dietary fat, protein, and carbohydrate Pancreatic insufficiency due to cystic fibrosis, pancreatitis, pancreatectomy Taken with every meal may increase incidence of gout... 

Patients most often requiring provision of digestive enzymes include (hose with chronic pancreatitis, mostly secondary to alcoholism, children and adults with cystic fibrosis, and patients with pancreatic carcinomas (fig. 12). In addition to these conditions, pancreatic enzymes are sometimes used because of their analgesic effect for chronic pancreatitis [65]. 

Q14 Cystic fibrosis patients are usually prescribed pancreatic enzymes to aid digestion. Which enzymes are likely to be included in these preparations ... 

Q10 Pancreatic insufficiency occurs in approximately 80% of cystic fibrosis patients. There is a marked reduction in the water, electrolyte and enzyme content of pancreatic secretion. Because of deficient digestive enzymes, there is inadequate digestion and absorption of nutrients and some nutritional deficiency occurs. 

Qll Pancreatic enzyme secretion is greatly reduced in cystic fibrosis. Relative or total absence of pancreatic lipase results in failure of fat digestion. The undigested fat is not absorbed, remains in the intestine and is excreted in relatively large quantities. The faeces are pale, bulky, smell unpleasant, float in water and are difficult to flush. This condition is called steatorrhoea. 

Patients with cystic fibrosis secrete very viscous mucus in the lung and suffer repeated lung infections. The pancreas is also affected and patients are deficient in pancreatic enzymes this reduces digestion and absorption of nutrients, so affecting growth. 

Since cystic fibrosis patients lack digestive enzymes, enzyme preparations containing amylase, lipase and proteases are prescribed in order to improve intestinal absorption of nutrients. 

The answer is a. (Murray, pp 627-661. Scriver, pp 3897-3964. Sack, pp 121-138. Wilson, pp 287-320.) Vitamins A, D, E, and K are all fat-soluble. The physical characteristics of fat-soluble vitamins derive from the hydrophobic nature of the aliphatic chains composing them. The other vitamins listed are water-soluble, efficiently administered orally, and rapidly absorbed from the intestine. Fat-soluble vitamins must be administered intramuscularly or as oral emulsions (mixtures of oil and water). In intestinal disorders such as chronic diarrhea or malabsorption due to deficient digestive enzymes, fat-soluble vitamins are poorly absorbed and can become deficient. Supplementation of fat-soluble vitamins is thus routine in disorders like cystic fibrosis (219700), a cause of respiratory and intestinal disease that is the likely diagnosis in this child. 

D. Because cystic fibrosis leads to pancreatic damage and diminution of the abihty to secrete HCO3 and pancreatic digestive enzymes with the result that fat and protein are absorbed poorly. Retinol is a fat-soluble vitamin that must be absorbed along with lipid micelles other fat-soluble vitamins are E, D, and K. The other vitamins hsted are water-soluble and their absorption is not significantly affected. 

Corn seed-derived dog gastric lipase is under development by Meristem Therapeutics for the treatment of gastric lipase deficiency (steatorrhea), neonatal deficient pancreatic function, or when pancreatic function is compromised by diseases such as cystic fibrosis or chronic alcoholism [159]. The absence of gastric lipase in steatorrhea patients prevents the digestion of food lipids. Pre-term infants have difficulty digesting lipids and, unless they are breast-fed, have no access to this enzyme. 

Pancreatin, a pancreatic enzyme, is indicated in exocrine pancreatic secretion insufficiency, as a digestive aid in cystic fibrosis, steatorrhea, and for other disorders of fat metabolism secondary to insufficient pancreatic enzymes. 

Patients with cystic fibrosis, such as Sissy Fibrosa, have a genetically determined defect in the function of the chloride channels. In the pancreatic secretory ducts, which carry pancreatic enzymes into the lumen of the small intestines, this defect causes inspissation (drying and thickening) of pancreatic exocrine secretions, eventually leading to obstruction of these ducts. One result of this problem is the inability of pancreatic enzymes to enter the intestinal lumen to digest dietary proteins. 

Also some drug formulations contain more than one enzyme. For example, pancrelipase or Zymase contains amylase (for processing starch), lipase (for breaking down fats), and chymotrypsin (a protease) in various combinations. This protein mixture is isolated from porcine or bovine pancreatic tissues and is sometimes called pancreatin. This enzyme cocktail is sold to help digestion in people who do not produce enough of their own digestive enzymes. Such a condition can arise due to pancreatic disfunction caused by pancreatitis, cystic fibrosis, or pancreatic cancer. 

A study in heart-lung transplant patients with cystic fibrosis found that they needed almost five times the oral dose of ciclosporin of other patients, confirming other studies in these patients that had shown a very much reduced bioavailability of oral ciclosporin. This is probably a reflection of the generally poor digestion and absorption in cystic fibrosis patients. The addition of pancreatic enzymes (Creori) was not found to improve this poor ciclosporin bioavailability. No adverse effects were reported. ... 

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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