Digestive system dysfunction in Cystic Fibrosis: Challenges for nutrition therapy
Li, Li Somerset, Shawn
Li, Li
Somerset, Shawn
Author
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
Date
2014
Type
Journal article
Journal
Digestive and Liver Disease
Book
Volume
46
Issue
10
Page Range
865-874
Article Number
ACU Department
School of Education
Faculty of Education and Arts
Faculty of Education and Arts
Collections
Relation URI
Source URL
Event URL
Open Access Status
Open access
License
CC BY-NC-ND 4.0
File Access
Open