Correction of Linoleic Acid Deficiency in Cystic Fibrosis

Abstract

ABSTRACT. To identify evidence of essential fatty acid deficiency, we screened 64 patients with cystic fibrosis by analyzing total lipid extracts from plasma. Forty-three had an abnormal linoleate (18:2) level (less than 26%). Thirteen deficient patients (aged 10-24 yr) ingested for 1 yr 7% of their total calories as linoleate derived from a daily supplement of Microlipid. Five deficient patients (aged 10-37 yr) served as controls. Plasma and erythrocyte fatty acid composition were monitored by gas chromatography of total lipid extracts seven times during the twelve month period. Prostaglandins E2 and FZa and their 15 keto 13, 14 dihydrometabolite, 6-keto F,,, and thromboxane B2 were measured by radioimmunoassay. Sweat tests, oxygen saturation, growth indices, clinical severity scores, compliance, and possible side effects from taking Microlipid were followed. Results showed that oral supplementation with Microlipid can significantly increase plasma and erythrocytes %18:2. One compliant patient died during the study and had normal tissue 18:2 levels. Nine of 13 patients gained more weight while taking Microlipid than in the previous year. No significant changes in sweat electrolytes, clinical scores, or oxygen saturation were found during the study year. Prostaglandin metabolites prostaglandin E2 showed an upward trend in supplemented patients, compared to controls. Prostaglandin FZa remained unchanged over 1 yr but showed a trend significantly downward over the final 6 months in supplemented patients. We conclude that linoleate deficiency can be corrected with daily Microlipid supplements and that correction may alter prostaglandin metabolism (Pediatr Res 20: 36-41, 1986) Abbreviations CF, cystic fibrosis 18:2, linoleate PGM, 13,14-dihydro-15-PGFza PG, prostaglandin EFA, essential fatty acid RBC, red blood cells Several workers (1 -3) have demonstrated markedly decreased concentration of essential fatty acid (EFA) in plasma from CF patients. The major changes include a decreased level of the predominant EFA, linoleate (1 8:2), and increased palmitoleate

Temporal Associations Among Energy Intake, Plasma Linoleic Acid, and Growth Improvement in Response to Treatment Initiation After Diagnosis of Cystic Fibrosis

“…19 Another study by van Egmond et al 25 showed better growth among infants who had CF and consumed a predigested formula that contained high LA (12% of energy) compared with those who consumed a comparable formula with lower LA (7% of energy), despite a lower intake of total energy in the former group. In support of these findings, studies of oral supplementation by Mischler et al 24 In view of the findings described above, we hypothesized that sustained high energy intake (1EN) and normal plasma essential fatty acid status are critical determinants of treatment responsiveness. Data from children who have CF and are enrolled in the Wisconsin CF Neonatal Screening Project 20,27 were used to examine this hypothesis for the period of 2 years after CF diagnosis.…”

“…23 In addition to optimizing energy intake, normalizing plasma essential fatty acid status was reported to be associated with better growth in children with CF. 19,[24][25][26] Low plasma linoleic acid (LA) concentrations and essential fatty acid deficiency are evident even among infants whose CF is diagnosed before 6 weeks of age via neonatal screening, with 54% having low plasma LA and 27% having abnormal triene/tetraene ratio. 18 Plasma LA concentration was shown to be correlated positively with growth in children whose CF was diagnosed before 3 months and followed up to 12 years of age.…”

Supplemental Calories Improve Essential Fatty Acid Deficiency in Cystic Fibrosis Patients

“…Results were variable but most showed minimal (7,35) or no (36) improvement in EFA status; however, caloric intakes, when reported, were below the RDA for control subjects (7). Improvements of plasma linoleic acid was observed in CF patients when dietary supplementation was directed at increasing calories as well as linoleic acid (9,37). We attribute the improvement in the EFA status of our CF patients to an improved caloric intake in the Dresence of a linoleic acid intake in excess of the RDA of 3% of total calories.…”

Normal phase liquid chromatography–electrospray ionization tandem mass spectrometry analysis of phospholipid molecular species in blood mononuclear cells: application to cystic fibrosis

“…Many abnormalities were subsequently discovered [4,5] and different oral [6,7] or parenteral [8,9] lipid supplements were administered to CF subjects in order to correct the defects [10,11]. Early studies attributed these abnormalities only to fat malabsorption; afterwards, the existence of an association between the basic defect and abnormal metabolism of essential fatty acids in CF was hypothesized [4,12].…”