Termination of strict diet in phenylketonuria: neurophysiological, psychological and biochemical studies

While the positive effect of early dietary treatment on the intellectual development of patients with phenylketonuria (PKU) (McKusick 26160) has been clearly established, controversy exists regarding the termination of the phenylalanine-restricted diet in PKU-children. A strict dietetic scheme poses serious problems especially during school age and it may elicit psychosocial abnormalities in the children as well as in their families. This scheme should therefore be abandoned when development of recognizable CNS dysfunction is no longer to be expected.