Nutritional Management in Phenylketonuria

Abstract

The dietary treatment of phenylketonuria (PKU) as followed by the Children's Hospital Research Foundation, Cincinnati, is based on the creation of a balance between the essential amino acids in the current low phenylalanine protein substitute, the major source of dietary protein, and the phenylalanine content of all other foods included in the diet. Guidelines for initiation of dietary treatment in newly diagnosed phenylketonuric infants are outlined. Continuous administration of dietary therapy is suggested to meet nutritional demands for phenylalanine and protein in the growing child. This method has been used for over three years, and results have shown that it is possible to maintain normal physical and mental development simultaneously with serum phenylalanine levels of 5 to 10 mg/100 ml.