Oral correction of essential fatty acid deficiency in cystic fibrosis.

A combination of pancreatic insufficiency and inadequate caloric intake may produce essential fatty acids (EFA) deficiency in patients with cystic fibrosis. Seventy-five percent of the adolescents and young adults with poor weight gain in our clinic were EFA-deficient by total plasma linoleic acid criteria. Twenty of these patients were placed on an oral hyperalimentation regimen containing 230% of calories required for basal energy expenditure, 40% as fat. Forty percent of these (8/20) achieved normal EFA levels on this diet. Eight of the nonresponding patients were given an additional 5% of their caloric intake as linoleic acid monoglyceride. All who maintained caloric intake achieved normal EFA levels. Normalization of EFA levels was associated with a number of clinical benefits including increase in weight and activity and, in five teenage girls, regulation of menses. The 16 control patients who received standard pancrelipase therapy and nutritional supplements remained fatty acid deficient. We conclude that oral hyperalimentation can restore EFA levels in cystic fibrosis patients if adequate calories are available to provide energy needs.