Digestive system dysfunction in Cystic Fibrosis: Challenges for nutrition therapy

Journal article


Li, Li and Somerset, Shawn. (2014). Digestive system dysfunction in Cystic Fibrosis: Challenges for nutrition therapy. Digestive and Liver Disease. 46(10), pp. 865 - 874. https://doi.org/10.1016/j.dld.2014.06.011
AuthorsLi, Li and Somerset, Shawn
Abstract

Cystic fibrosis can affect food digestion and nutrient absorption. The underlying mutation of the cystic fibrosis trans-membrane regulator gene depletes functional cystic fibrosis trans-membrane regulator on the surface of epithelial cells lining the digestive tract and associated organs, where Cl− secretion and subsequently secretion of water and other ions are impaired. This alters pH and dehydrates secretions that precipitate and obstruct the lumen, causing inflammation and the eventual degradation of the pancreas, liver, gallbladder and intestine. Associated conditions include exocrine pancreatic insufficiency, impaired bicarbonate and bile acid secretion and aberrant mucus formation, commonly leading to maldigestion and malabsorption, particularly of fat and fat-soluble vitamins. Pancreatic enzyme replacement therapy is used to address this insufficiency. The susceptibility of pancreatic lipase to acidic and enzymatic inactivation and decreased bile availability often impedes its efficacy. Brush border digestive enzyme activity and intestinal uptake of certain disaccharides and amino acids await clarification. Other complications that may contribute to maldigestion/malabsorption include small intestine bacterial overgrowth, enteric circular muscle dysfunction, abnormal intestinal mucus, and intestinal inflammation. However, there is some evidence that gastric digestive enzymes, colonic microflora, correction of fatty acid abnormalities using dietary n − 3 polyunsaturated fatty acid supplementation and emerging intestinal biomarkers can complement nutrition management in cystic fibrosis.

Keywordscystic fibrosis; gut microbiota; malabsorption; maldigestion
Year2014
JournalDigestive and Liver Disease
Journal citation46 (10), pp. 865 - 874
PublisherElsevier Inc.
ISSN1878-3562
Digital Object Identifier (DOI)https://doi.org/10.1016/j.dld.2014.06.011
Open accessOpen access
Page range865 - 874
Research GroupSchool of Allied Health
Publisher's version
License
Place of publicationUnited States of America
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