FATTY-ACID METABOLISM IN PHENYLKETONURIA

Children treated for phenylketonuria (PKU) have a low intake of whole animal foods. Consequently, the dietary intake of long-chain polyunsat urated fatty acids (PUFA) is just a few milligrams per day, mostly rep resented by arachidonic acid (AA). In a consecutive series of studies, we assessed in treated PKU children their long-chain PUFA status, the AA-related eicosanoid synthesis and the effects of specific PUFA supp lementations. We found that the good compliance with the dietary regim en negatively influences the long-chain PUFA status and serum eicosano id release from platelets. Supplementation with either marine or black currant oils modifies the long-chain PUFA status of PKU children witho ut approaching the fatty acid pattern of a healthy control population. Good-compliant PKU patients have diet-related, low levels of circulat ing long-chain PUFA, whose clinical and functional consequences deserv e further investigation. The effects of dietary supplementations with long-chain PUFA of both the n-6 and n-3 series should be carefully eva luated.