Nutritional approach to PKU diet

Phenylketonuria (PKU) represents an ever changing challenge for the nutritionist. The avoidance of unpermitted foods in infants leads to the exclusion of nutrients naturally occurring only in whole animal foods and recently identified as essential or semi-essential for adequate growth and development. Moreover, the deranged amino acid metabolism could cause abnormalities in many physiological processes. As a result, body and skeletal growth and neurodevelopment should be carefully assessed in treated PKU children to improve dietary intervention. Minerals, trace elements, taurine and carnitine have been studied for possible deficiencies in PKU. More recently, emphasis has been laid on the role of long chain polyunsaturated fatty acids (PUFA) in human growth and development. The dietary supplementation of PKU with long-chain PUFA-enriched oils should be carefully assessed not only biochemically but also for the possible functional consequences in nursing PKU infants and in cases of maternal PKU.