Essential fatty acids in cystic fibrosis.

The nature of the metabolic defect in cystic fibrosis (CF) has, to 
date, not been elucidated. Elliott reported that intravenous administration of soybean 
oil and lecithin to children suffering from CF caused their sweat 
sodium concentration to decrease towards normal values. This prompted us to 
investigate the fatty acid spectra of the various serum lipid classes 
of children with CF. We have found that affected children are 
deficient in essential fatty acids. The data suggest another approach to 
the study of the metabolic defect in cystic fibrosis. There is 
an apparent deficiency in essential fatty acids, and a marked reduction 
in serum vitamin E levels has also been reported in CF. 
These conditions might be partially corrected by intravenous injection, or possibly 
feeding of, lipids containing essential fatty acids with concomitant administration of 
vitamin E. The observed deficiency in essential fatty acids may be 
produced by a reduced ability to absorb these acids from the 
diet and this, in turn, may result in defects in membrane 
structure or stability. Reduced levels of vitamin E in CF may 
reflect a reduced requirement for this vitamin because of the lower 
levels of essential fatty acids. It is also possible that the 
observed deficiency in serum levels of essential fatty acids may lead 
to less than normal production of prostaglandins. 