Pancreatic enzyme deficiency

Impaired degradation of R-proteins in intestine Pancreatic enzyme deficiency ... 

The clinical abnormalities related to the gastrointestinal tract are not life-threatening and can be treated. In newborns with CF, intestinal obstruction (meconium ileus) can occur in 10-20% of cases due to failure of digestion of intraluminal contents due to lack of pancreatic enzymes in utero. Exocrine pancreatic enzyme deficiency is present from birth affecting both lipid and protein digestion. In general, carbohydrate digestion is not severely impaired. 

Because of the lipase deficiency, fat-soluble vitamin (A, D, E, and K) deficiencies may occur. Whether lipase activity or bile acids (e.g., in micelle formation) are involved in fat-soluble vitamin absorption with steatorrhea is unclear. Vitamin and zinc deficiencies also may occur as aresult of pancreatic enzyme deficiency. Although pancreatic involvement is predominantly exocrine in nature, insulin deficiency with glucose intolerance also occurs in CF patients, especially as they advance in age. Carbohydrate intolerance is characterized by low insulin concentrations and enhanced peripheral sensitivity to insulin but not by the presence of islet cell or anti-insulin antibodies. Carbohydrate intolerance in CF is not usually associated with the ketosis as commonly occurs in type 1 diabetes. This complication involves an increase in the number of insulin receptors with decreased affinity for insulin. Despite a concomitant increase in tissue affinity for insulin, 8% of CF children over 12 years of age require insulin therapy. 

TKErap cat (veD In replacement therapy in pancreatic enzyme deficiency, in preparation of "predigested protein foods,... 

The client s symptoms should indicate hyperglycemia to the nurse, not pancreatic enzyme deficiency. 

The presence or absence of pancreatic enzymes can only be satisfactorily decided by intraduodenal intubation and direct examination of samples of small intestinal contents after the administration of a suitable stimulus to pancreatic secretion (Fll). It is not sufficient to look at one enzyme only, such as trypsin, since a specific deficiency of lipase can occur (Sll). Assessment of the degree of hydrolysis of fat in the stools is quite unreliable as a guide to pancreatic enzyme activity (CIO). 

Bile salt deficiency must also be directly studied. It may occur in the absence of obstruction or obvious liver disease (R7). The majority of patients with one form or another of the sprue syndrome will be found to have pancreatic enzymes and bile salts within the normal range. Pancreatic enzymes are absent or markedly deficient in patients with pancreatogenous malabsorption syndrome (B17, F13). It is surprising how frequently this necessary step in differential diagnosis is omitted. 

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. 

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. 

Isolated enzyme deficiencies, lipase, colipase, enterokinase Pancreatic insufficiency secondary to other disorders Celiac disease... 

The effectiveness of pancreatic enzyme supplementation in treating malabsorption is measured by improvement in body weight and stool consistency or frequency. The 72-hour stool test for fecal fat may be used when there is concern regarding the adequacy of treatment. Serum uric acid and folic acid concentrations should be monitored yearly in patients prone to hyperuricemia or folic acid deficiency. Blood glucose must be closely monitored in the diabetic patient. 

IVLE should be used in patients with liver failure only to prevent essential fatty acid deficiency when initial serum triglyceride concentrations exceed 300 mg/dL. If serum triglyceride concentrations are low or normal, IVLE should be used as a calorie source. Monitoring serum triglyceride concentration and FFA oxidation (not available in all facilities) to ensure that lipid is both cleared and oxidized appropriately has been suggested. Triglyceride concentrations are the only available marker in most clinical practices at this time. Oral MCTs have been used occasionally with success because they do not require pancreatic enzymes or micelle formation before absorption. However, these products do not provide essential fatty acids. 

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