Polyunsaturated fatty acid deficiency during dietary treatment of very long-chain acyl-CoA dehydrogenase deficiency. Rescue with soybean oil

Nutritional management of very long-chain acyl-CoA dehydro"genase (VLCAD) d eficiency is based on the avoidance of fasting and substitution of medium-c hain triglycerides for long- and very long-chain triglycerides. We report t wo cases of this disease, which developed omega -6 essential fatty acid def iciency after three and five months from the beginning of nutritional thera py (SHS product: Monogen). This alteration could be especially dangerous in these patients owing to their possible susceptibility to the development o f pigmentary retinopathy. The incorporation of linoleic acid as 3-4% of tot al caloric intake supported as soybean oil ameliorates this deficiency. We wish to remark on this early complication in the nutritional management of VLCAD deficiency and the possibility of rescue by the incorporation of soyb ean oil into the diet.