Dietary Essential Fatty Acids in Cystic Fibrosis

Abstract

Analyses of serum lipids of children with cystic fibrosis (CF) have indicated a deficiency in essential fatty acids (EFA). In view of a report that intravenous administration of soybean oil emulsions normalized sweat sodium values in CF children, we studied the effects of orally administered essential fatty acids (as corn oil) for one year. Some improvement was noted in all patients, but no one consistent factor predominated. However, arachidonic acid, which was found only in trace amounts or was absent in sera of all children with CF before the clinical trial, was increased significantly in the sera after the oral administration of EFA for one year. Other findings included a significant decrease in sweat sodium and T3 in most patients.

Linoleic Acid Absorption from Lipid Supplements in Patients with Cystic Fibrosis with Pancreatic Insufficiency and in Control Subjects

“…In addition , the enzyme suppleme nts co nsumed often do not complete ly correct the malabs orptio n of fat s in th ese pat ients (9,11,27) . As mentioned pre viou sly, the result s of long-term supplementat ion with lipids hav e been quit e variable (3,(14)(15)(16)(17)(18)(19)(20), suggesti ng th at mo re information is needed about the question of lipid ab sorption and metabolism in CF patients with pancreatic insufficien cy. Possible explanations for the se variable results include: pati ents (a) are not tak ing the supplement s ; (b) are not taking their ex ogenous enzymes -----'-----'--- with the supplements ( 15); (c) are not being given adequate linoleic acid from the suppl ements; (d) are not consuming enough calo ries and therefore utilize the linoleic acid for energy (27,28); or (e) have some defect in fatt y acid metabolism (2,3), perh ap s related to altered liver metabolism (3,II ,29).…”

“…Corn oil , safflower oil , linoleic acid monoglyce ride, and vario us lipid infu sion s ha ve all been used (3,14-1 8) as a sourc e of linoleic ac id for longterm supplementation trial s , usuall y lasting 1 year. Results have been variable , but mo st have shown minimal (3,16) or no (17,18) improvement in the linoleic acid sta tus of the CF patients . However, impro ved to normalized levels of plasma linolei c ac id have been ob served in supplementation studies condu ct ed w it h maj o r effo rts to wards c o mplia nc e (19,20) .…”

Nutrition in Cystic Fibrosis—Some Notes on the Fat Recommendations

“…ENaC are expressed in the kidney, and the renal excretion of sodium was restored after long-term LA supplementation [67]. Sodium excretion in sweat was also decreased after long-term LA supplementation [59,74,117]. Other transporters and membrane-bound activities might also be influenced, since the Na(+)-K+ ratio in erythrocytes was normalized by regular LA supplementation [118].…”

Relationships Between Pulmonary Function and Plasma Fatty Acid Levels in Cystic Fibrosis Patients

“…Summary: Nineteen patients with cystic fibrosis (CF) were studied to determine whether plasma fatty acids correlated with severity of their lung disease as assessed by pulmonary function testing, Results were compared with 19 normal subjects of similar age and sex, Linoleic acid content of all lipid fractions was significantly lower in CF patients than controls including cholesterol ester fraction (CF 31%, control 50%, p < 0,001), triglyceride fraction (7,6 to 16,6%, p < 0,001), and phospholipid fraction (13.9 to 21.7%, p < 0.001). Mean 20:3w9/20:4w6 ratio for CF patients was higher in all lipid classes and was suggestive of essential fatty acid deficiency, Correlations Decreased pancreatic enzyme secretions in cystic fibrosis (CF) (I) may lead to malnutrition, and thus it is not surprising that there have been several reports of altered fatty acid distribution in several plasma lipid classes (2)(3)(4)(5)(6)(7). In general these reports have documented elevated levels of palmitic (16:0), palmitoleic (16: I), and oleic (18: I) acids and decreased linoleic and arachidonic acid levels.…”

“…Intervention studies designed to correct EFA status in CF patients have been reported with conflicting results. Attempts to correct EFA levels by intravenous fat emulsion (Intralipid) (13,15,16) or by oral supplementation with corn or sunflower oils (7,17,18) have mostly failed either to raise the level of EFA in serum or to detect improvement in a clinical condition.…”