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Mechanisms of Lipid Malabsorption in Cystic Fibrosis: the Impact of Essential Fatty Acids Deficiency

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Publisher Biomed Central
Date 2005 May 5
PMID 15869703
Citations 17
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Abstract

Transport mechanisms, whereby alimentary lipids are digested and packaged into small emulsion particles that enter intestinal cells to be translocated to the plasma in the form of chylomicrons, are impaired in cystic fibrosis. The purpose of this paper is to focus on defects that are related to intraluminal and intracellular events in this life-limiting genetic disorder. Specific evidence is presented to highlight the relationship between fat malabsorption and essential fatty acid deficiency commonly found in patients with cystic fibrosis that are often related to the genotype. Given the interdependency of pulmonary disease, pancreatic insufficiency and nutritional status, greater attention should be paid to the optimal correction of fat malabsorption and essential fatty acid deficiency in order to improve the quality of life and extend the life span of patients with cystic fibrosis.

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References
1.
Holman R . The ratio of trienoic: tetraenoic acids in tissue lipids as a measure of essential fatty acid requirement. J Nutr. 1960; 70(3):405-10. DOI: 10.1093/jn/70.3.405. View

2.
Sarfeh I, Beeler D, TREBLE D, BALINT J . Studies of the hepatic excretory defects in essential fatty acid deficiency. Their possible relationship to the genesis of cholesterol gallstones. J Clin Invest. 1974; 53(2):423-30. PMC: 301485. DOI: 10.1172/JCI107576. View

3.
Clark S . Chylomicron composition during duodenal triglyceride and lecithin infusion. Am J Physiol. 1978; 235(2):E183-90. DOI: 10.1152/ajpendo.1978.235.2.E183. View

4.
Smith L, Lacaille F, Lepage G, Ronco N, Lamarre A, Roy C . Taurine decreases fecal fatty acid and sterol excretion in cystic fibrosis. A randomized double-blind trial. Am J Dis Child. 1991; 145(12):1401-4. DOI: 10.1001/archpedi.1991.02160120069022. View

5.
Bosch V, Otayek M . Lipid transport across the intestinal epithelial cell. Effect of colchicine. Biochim Biophys Acta. 1976; 431(2):297-302. DOI: 10.1016/0005-2760(76)90150-8. View