The essential fatty acid status in phenylketonuria patients under treatment

Phenylketonuric patients are on a special diet that lacks certain essential fatty acids. This study evaluates the essential fatty acid status of a gro up of phenylketonuric patients in the Netherlands undergoing dietary treatm ent. To this end, the essential fatty acid status of nine phenylketonuria p atients was studied. On the basis of age and gender, two control subjects w ere selected far each patient The essential fatty acid composition of dupli cate food portions and the essential fatty acid status of plasma and erythr ocytes were analyzed. Phenylketonuria subjects had a different essential fa tty acid profile from their peers, especially concerning the n-3 fatty acid s. N-6 and n-3 fatty long-chain polyenes were hardly consumed by phenylketo nuria subjects, in contrast to the control subjects. Linoleic acid, on the other hand, was consumed in significantly higher amounts by phenylketonuria subjects and made up about 40% of their daily fat consumption. The essenti al fatty acid consumption pattern of the phenylketonuria subjects is mirror ed by the essential fatty acid concentrations in blood. The essential fatty acid status of the phenylketonuric diet should be improved in order to pre vent deficiency in n-3 fatty acids. (C) Elsevier Science Inc. 2000. All rig hts reserved.