Cystic fibrosis pancreas

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,... 

Cystic fibrosis (MIM 219700) CFTR(C - channel) Lungs, 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. 

Cystic fibrosis (CF) is a hereditary disease of abnormal fluid secretion. It affects cells of the exocrine glands, such as intestine, sweat glands, pancreas, reproductive tract, and especially the respiratory tract. The disease affects about 1 in 2500 infants of the Caucasian population to varying degrees of seriousness. Patients produce thickened mucus that is difficult to get out of the airway. This leads to chronic lung infection, which progressively destroys pulmonary function. 

Parenteral Anticoagulant-induced prothrombin deficiency hypoprothrombinemia secondary to conditions limiting absorption or synthesis of vitamin K (eg, obstructive jaundice, biliary fistula, sprue, ulcerative colitis, celiac disease, intestinal resection, cystic fibrosis of the pancreas, regional enteritis) drug-induced hypoprothrombinemias due to interference with vitamin K metabolism (eg, antibiotics, salicylates) prophylaxis and therapy of hemorrhagic disease of the newborn. 

Cystic fibrosis (CF)—genetic disease affecting the lungs and pancreas caused by a mutation of a gene for chloride transport protein. It was targeted by early efforts at gene therapy, but without success. 

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. 

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. 

Abnormalities in protein digestion In individuals with a deficiency in pancreatic secretion (for example, due to chronic pancreatitis, cystic fibrosis, or surgical removal of the pancreas), the digestion and absorption of fat and protein is incomplete. This results in the abnormal appearance of lipids (called steatorrhea, see p. 175) and undigested protein in the feces. 

Gibson, L.E., and R.E. Cooke. 1959. A test for concentration of electrolytes in sweat in cystic fibrosis of the pancreas utilizing pilocarpine by iontophoresis. Pediatrics Evanston) 23 545. 

W. 5. Cbernick and G, J. Butau Composition of tracheobronchial secretions m cystic fibrosis oF the pancreas a nd broochlectuk Pediatries 24 739-743 (1959). 

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. 

A disease that results from a decrease in fluid and salt secretion by a transport protein referred to as the cystic fibrosis transmembrane conductance regulator (CFTR). As a result of this defect, secretion from the pancreas is blocked, and heavy, dehydrated mucus accumulates in the lungs, leading to chronic lung infections. 

Annular pancreas, pancreas divisum, pancreatic hypoplasia and agenesis, heterotopic pancreas Inherited syndromes affecting the pancreas Cystic fibrosis... 

Miscellaneous Causes of Diabetes Mellitus These include diseases of the exocrine pancreas (e.g., cystic fibrosis, hemochromatosis, pancreatitis) and drug-induced causes (e.g., thiazides, glucocorticoids). 

CFTR discovered 20 years ago, is a cAMP-activated chloride channel, acting as an ATP-dependent pump with ATPase activity, expressed in epithelia in the lung, intestine, pancreas and other tissues, where it facilitates tran-sepithelial fluid transport. In the intestine, CFTR provides the major route for chloride secretion in certain diarrheas. Mutations in CFTR cause the hereditary disease cystic fibrosis, where chronic lung infection and deterioration in lung function cause early death. 

Recently, Cl" channels have been discovered. These channels have no sequence relationship to the voltage-gated Na, and Ca channels. One such channel is involved in the disease, cystic fibrosis. In this disease, regulation of the channel is defective. The altered function of the channel in epithelia causes elevated levels of sodium and chloride ions in sweat and, through unknown processes, the accumulation of mucus in the respiratory tract and failure of exocrine secretion in glands, such as the pancreas. Blockage of airways leads to chronic lung infections that, with other effects of the Cl" transport deficiency, can be fatal. 

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