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.
Highlights
Cystic fibrosis (CF) is the most common autosomal recessive genetic disease observed in the Caucasian population, affecting about 1 in 2,500 newborns
It is difficult to reconcile the failure of appropriate pancreatic enzyme replacement therapy with the persistent fat malabsorption
Since mutations in CFTR result in impaired intracellular pH organelle, glycosylation and sialylation in mammalian cells, it is possible that disturbances in intestinal CF lipid transport may be associated with cause-related changes in the second step of fat absorption, i.e. the intracellular phase leading to lipolytic product uptake and esterification, apolipoprotein synthesis and processing, and nascent lipoprotein assembly and secretion following the fusion of Golgi vesicles with the basolateral plasma membrane
Summary
Cystic fibrosis (CF) is the most common autosomal recessive genetic disease observed in the Caucasian population, affecting about 1 in 2,500 newborns. A membrane lipid imbalance plays a role in the phenotype expression in CFTR knock-out mice and feeding these animals with DHA has normalized disease-related changes that occur in epithelial cells and intestinal mucosa [63]. Http://www.nutritionandmetabolism.com/content/2/1/11 ical effects of EFA supplementation vary among studies: linoleic acid increases weight for height in CF children [19,22] and an improvement in pulmonary function is observed with n-3 FA intake. This valuable effect is not reported in all studies. These lipid digestive and absorptive steps will be discussed chronologically
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