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
Authors | Li, Li and Somerset, Shawn |
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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. |
Keywords | cystic fibrosis; gut microbiota; malabsorption; maldigestion |
Year | 2014 |
Journal | Digestive and Liver Disease |
Journal citation | 46 (10), pp. 865 - 874 |
Publisher | Elsevier Inc. |
ISSN | 1878-3562 |
Digital Object Identifier (DOI) | https://doi.org/10.1016/j.dld.2014.06.011 |
Open access | Open access |
Page range | 865 - 874 |
Research Group | School of Allied Health |
Publisher's version | License |
Place of publication | United States of America |
https://acuresearchbank.acu.edu.au/item/862xz/digestive-system-dysfunction-in-cystic-fibrosis-challenges-for-nutrition-therapy
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