Persistence of Essential Fatty Acid Deficiency in Cystic Fibrosis Despite Nutritional Therapy

Abstract

ABSTRACT:To study the evolution of plasma fatty acid composition of patients with cystic fibrosis (CF) in relation to nutritional status, pancreatic function, and development of CF-related liver disease (CFRLD) and diabetes mellitus, 24 CF pediatric patients with stable pulmonary disease were studied before and after an approximate period of 8 y. Nutritional status, pulmonary function, pancreatic function, and presence of CFRLD or diabetes mellitus were recorded. Results were compared with data obtained in 83 healthy children. Patients with CF have significantly lower linoleic acid (LA), docosahexaenoic acid (DHA), lignoceric acid, and LA ϫ DHA product and higher oleic acid, mead acid, dihomo-␥-linoleic acid, and docosapentaenoic acid (DPA). Comparison of samples taken at first and second studies revealed a significant decrease in LA levels and lignoceric acid associated with a significant increase in dihomo-␥-linoleic acid levels. Patients with CFRLD showed significantly higher mead acid/arachidonic acid ratio and lower total 6 polyunsaturated fatty acids content. There was no relation of plasma fatty acids composition with pancreatic function, pulmonary function, or diabetes mellitus. Follow-up of patients with CF shows that essential fatty acids deficiency, particularly in LA and DHA content, persisted unmodified along time despite an adequate nutritional therapy. Future studies after supplementation with 3 polyunsaturated fatty acids should be undertaken. (Pediatr Res 66: 585-589, 2009)

The effects of ivacaftor on CF fatty acid metabolism: An analysis from the GOAL study

“…At baseline, we observed fatty acid levels that were similar to what has been previously reported, including low levels of DHA as well as elevated levels of mead acid and palmitoleic acid (3, 21, 22). Additionally, despite a decrease in mead acid levels in the overall cohort, we did not observe a significant decrease in the triene: tetraene (T:T) ratio because there was also a decrease in arachidonic acid levels.…”

“…Additionally, despite a decrease in mead acid levels in the overall cohort, we did not observe a significant decrease in the triene: tetraene (T:T) ratio because there was also a decrease in arachidonic acid levels. An elevated T:T ratio is used clinically as a marker of essential fatty acid deficiency and it has previously been reported as being elevated in individuals with CF (22, 23); though, the etiology for fatty acid changes in individuals with CF is likely different than that of individuals with dietary essential fatty acid deficiency as for individuals with CF the fatty acid changes are likely related to up-regulation of enzymes of fatty acid metabolism by dysfunctional CFTR (5). …”

Inflammation in cystic fibrosis lung disease: Pathogenesis and therapy

“…In CF, there is an increase in AA and a decrease in DHA. There have been a few clinical trials of DHA supplementation in CF [141,142]. Unfortunately, the dose of DHA, study size, duration of treatment, and outcome measures vary widely.…”

Cystic Fibrosis-Related Oxidative Stress and Intestinal Lipid Disorders

“…However, various studies show the limited impact of this routine therapy on patients' clinical conditions. In fact, a negligible improvement in anthropometric measures, an increase in saturated FAs and monounsaturated FAs, an increase in DHA/AA ratio, and onset of diabetes-type hepatic and metabolic complications have been reported (34,49,77,108). Some authors also mention significantly low levels of vitamin E associated with significantly high levels of oxysterols in patients with pancreatic insufficiency or diabetes.…”

“…To this day, lipid disorders observed in CF patients remain a concern and still raise a host of questions. In fact, various studies show that despite the intake of gastroresistant pancreatic extracts associated with adequate dietary intake, OxS and FA abnormalities persist (108,159). A parallel plasma FA and ROS concentrations increase (161), given a rise to speculation about tolerance of the high-fat diet recommended for the nutritional care of patients.…”