Diagnosis and Treatment of Intestinal Malabsorption in Cystic Fibrosis

• Published in: Pediatric pulmonology 2006-01, Vol.41 (1), p.35-49
• Main Authors: Littlewood, James M., Wolfe, Susan P., Conway, Steven P.
• Format: Article
• Language: English
• Subjects: Adolescent; Adult; Biological and medical sciences; Child; Child, Preschool; cystic fibrosis; Cystic Fibrosis - complications; Enzymes - therapeutic use; Errors of metabolism; Exocrine Pancreatic Insufficiency - complications; Exocrine Pancreatic Insufficiency - therapy; Gastroenterology. Liver. Pancreas. Abdomen; Humans; malabsorption; Malabsorption Syndromes - diagnosis; Malabsorption Syndromes - etiology; Malabsorption Syndromes - therapy; Medical sciences; Metabolic diseases; Miscellaneous hereditary metabolic disorders; nutrition; Other diseases. Semiology; Pancreas - enzymology; Pancreas - metabolism; pancreatic enzymes; Pneumology; Stomach. Duodenum. Small intestine. Colon. Rectum. Anus; Treatment Outcome
• ISSN: 8755-6863; 1099-0496
• DOI: 10.1002/ppul.20286

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. Pediatr Pulmonol. © 2005 Wiley‐Liss, Inc.