Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024523 (malabsorption)
 7,319 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Intestinal malabsorption is severe and of early onset in virtually all people who have cystic fibrosis. The main cause is deficiency of pancreatic enzymes, but bicarbonate deficiency, abnormalities of bile salts, mucosal transport and motility, and anatomical structural changes are other contributory factors. Appropriate pancreatic replacement therapy will achieve normal or near normal absorption in many patients. It is important to identify both malabsorption and evidence of a pancreatic lesion in all patients who are to receive pancreatic enzymes. All who have evidence of fat malabsorption are deemed pancreatic insufficient and candidates for enzyme replacement therapy. Effective treatment should allow a normal diet to be taken, control symptoms, correct malabsorption and achieve a normal nutritional state and growth. The occurrence of fibrosing colonopathy in some patients receiving very high doses of those enzymes that have the copolymer Eudragit L30 D55 in their covering has resulted in guidelines in the UK to avoid dosages greater than the equivalent of 10,000 IU lipase/kg/day for all patients and also to avoid preparations containing this copolymer in children and adolescents. For patients not responding to 10,000 IU lipase/kg/day, review of adherence to treatment, change of enzyme preparation, variation of the time of administration and reduction in gastric acid may improve absorption. The importance of excluding other gastrointestinal disorders as a cause of the patient's symptoms and the need for early investigations, rather than merely increasing the dosage of enzymes, is stressed. With modern enzymes, adequate control of gastrointestinal symptoms and absorption can be achieved at dosages of 10,000 IU lipase/kg/day or only slightly more, and a normal nutritional state and growth rate maintained in most patients with cystic fibrosis.
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PMID:Control of malabsorption in cystic fibrosis. 1093 71

Intestinal malabsorption is severe and of early onset in virtually all people who have cystic fibrosis. The main cause is deficiency of pancreatic enzymes. Bicarbonate deficiency, abnormal bile salts, mucosal transport problems, motility differences, and anatomical structural changes are other contributory factors. Effective treatment should allow a normal to high-fat diet to be taken, control symptoms, correct malabsorption, and achieve a normal nutritional state and growth. Appropriate pancreatic enzyme replacement therapy will achieve normal or near-normal absorption in most people with cystic fibrosis. Early identification and treatment of intestinal malabsorption is critical to achieving optimal nutritional status. The occurrence of fibrosing colonopathy in a few patients on very high doses of those enzymes which have the copolymer Eudragit L30 D55 in their covering resulted in guidelines in the UK to avoid doses equivalent to more than 10,000 IU lipase per kg per day, and also to avoid preparations containing this copolymer in children and adolescents. For patients not responding to 10,000 IU lipase per kg per day review of adherence to treatment, change of enzyme preparation, variation in time of administration, and reduction in gastric acid may improve absorption. The importance of early investigation to exclude other gastrointestinal disorders as a cause of the patient's symptoms, rather than merely increasing the dose of enzymes, is stressed. With modern pancreatic enzymes in doses up to or only slightly in excess of 10,000 IU lipase per kg per day, adequate control of gastrointestinal symptoms and absorption can be achieved, and a normal nutritional state and growth rate maintained in most people with cystic fibrosis.
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PMID:Diagnosis and treatment of intestinal malabsorption in cystic fibrosis. 1628 83