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Mechanisms of lipid malabsorption in Cystic Fibrosis: the impact of essential fatty acids deficiency

N Peretti1 email, V Marcil1 email, E Drouin2 email and E Levy1 email

Department of Nutrition, CHU-Sainte-Justine, Université de Montréal, Montréal, Québec, Canada

Department of Pediatrics, CHU-Sainte-Justine, Université de Montréal, Montréal, Québec, Canada

author email corresponding author email

Nutrition & Metabolism 2005, 2:11doi:10.1186/1743-7075-2-11

Published: 3 May 2005

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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