Supplemental Calories Improve Essential Fatty Acid Deficiency in Cystic Fibrosis Patients

Abstract

ABSTRACT. Fatty acid composition of plasma lipids was analyzed in malnourished cystic fibrosis patients undergoing 6 months of nutritional rehabilitation. There were three males and five females (mean age 15.1 yr); five patients had pancreatic insufficiency. Nutritional rehabilitation in seven of eight patients was accomplished by nocturnal nasogastric infusion of a high-carbohydrate semisynthetic diet, in addition to daily meals. One patient received highenergy food supplements as snacks in addition to regular meals. All patients were moderately to severely malnourished on entry to the study and showed significant improvement over the 6 months in (3 f SE) energy intake (96 + 8.0 to 126 f 11% recommended daily allowance) and body composition (80 f 4 to 90 f 4% ideal body weight). Daily intakes of linoleic acid were not significantly different before or during nutritional rehabilitation either as an absolute amount (383 f 45 to 557 f 124 mg/kg/day) or as a percentage of total calories (4.50 f 0.40 to 4.73 f 0.14%). In comparison to the controls, the relative percentage of plasma cholesterol ester fatty acids of the CF patients on entry into the study showed a marked decrease of linoleic acid (52.7 f 1.0 versus 42.3 f 2.7%) with elevated palmitoleic (2.34 f 0.2 versus 5.64 2 0.7%) and oleic (18.7 f 1.0 versus 25.2 2 1.4%) acids; a pattern consistent with essential fatty acid deficiency. However, this pattern is not truly characteristic of a pure linoleic acid deficiency as the metabolites of linoleic acid were not decreased. After nutritional rehabilitation the linoleic acid concentration reached control values in the phospholipid and cholesterol ester plasma lipids in six of eight and five of eight patients, respectively. These findings indicate a low caloric intake is an important factor in determining the essential fatty acid status of cystic fibrosis patients and recovery of both the body composition and essential fatty acid deficiency can be accomplished by increasing the caloric intake to 150% of recommended daily allowances. (Pediatr Res 24: 353-356,1988) Abbreviations EFA, essential fatty acid CF, cystic fibrosis RDA, recommended daily allowance Many investigators have described a high incidence of biochemical EFA deficiency in CF patients (1-5). These patients

Effect of Administration of Gamma-Linolenic Acid on the Fatty Acid Composition of Serum Phospholipids and Cholesteryl Esters in Patients with Cystic Fibrosis

“…The observations on the fatty acid abnor malities in our CF group are generally in agreement with data reported by others [21][22][23][24][25][26][27][28][29][30][31][32], Linoleic acid in the CE fraction has been proposed as a good indicator for the degree of disturbance of the essential fatty acid status [27] as it makes up about half of the fatty acids in this fraction in normals. If this criterion is taken, disturbance of the essential fatty acid status of our CF group is rather mild com pared to several groups described in the litera ture.…”

Essential fatty acid deficiency in well nourished young cystic fibrosis patients

“…Both these parameters are used for diagnosing essential fatty acid de®ciency [14]. Such a de®ciency is well known in CF subjects [10,15,19,20,24,28,32,33,34] but unfortunately the nutritional status is usually not well described in these studies. As a whole, our young CF subjects were not undernourished and had a normal body composition by anthropometry and DEXA.…”

“…Those related to proteinenergy malnutrition are particularly compelling because of the impact on long-term prognosis [18]. Essential fatty acid de®ciency has very often been reported in CF patients [10,15,19,20,24,28,32,33,34], in particular those with protein-energy malnutrition [20]. It has been attributed to low fat diet [1,20], fat malabsorption [10,15,24], increased lipid turnover in cell membranes [33], defects in the desaturase activity [24,25,27], increased oxidation of fatty acids for energy source [28] and increased production of eicosanoids [38,39].…”

Plasma fatty acids and lipid hydroperoxides increase after antibiotic therapy in cystic fibrosis

“…2) fat malabsorption [3,4], 3) increased calorie supply to restore energy [5], 4) increased metabolism of EFA and overproduction of eicosanoids [6], 5) increased turnover of FAs in cell membranes [7], 6) increased peroxidation of polyunsaturated FAs [8], 7) defect of desaturase activity [3,9], and 8) specific genotype-linked basic defects [9][10][11].…”