ESSENTIAL FATTY-ACID DEFICIENCY IN WELL NOURISHED YOUNG CYSTIC-FIBROSIS PATIENTS

Essential fatty acid deficiency is well known in cystic fibrosis patie nts-but its pathogenesis remains unclear. It might be related to prote in-energy malnutrition which is a common feature of cystic fibrosis or to some specific defects in fatty acid metabolism. To avoid the delet erious effects of protein-energy malnutrition, this study assesses the plasma phospholipid fatty acid pattern in well nourished young cystic fibrosis subjects. Sixteen cystic fibrosis subjects aged 6.6-20.0 yea rs were studied and compared to 16 healthy controls matched for gender , age and nutritional status. Plasma phospholipids were separated by t hin layer chromatography and phospholipid fatty acid pattern was deter mined by gas liquid chromatography. Anthropometry and dual-energy X-ra y absorptiometry showed that lean body mass: fat-free mass and fat mas s were similar in the two groups. Nutritional inquiry showed higher in gestion of macronutrients by cystic fibrosis subjects than by controls . Plasma phospholipid palmitoleic acid and eicosatrienoic acid were hi gher, and by contrast linoleic acid and docosahexaenoic acid were lowe r in cystic fibrosis subjects than in controls. The ratio linoleic aci d/arachidonic acid was lower and the ratio eicosatrienoic acid/arachid onic acid was higher in cystic fibrosis subjects than in controls.Conc lusion Essential fatty acid deficiency is present in young cystic fibr osis subjects in the absence of protein-energy means that this: defici ency isis probably related to specific defects in in fatty acid metabo lism.