CHEMICAL AND CLINICAL OBSERVATIONS DURING TREATMENT OF CHILDREN WITH PHENYLKETONURIA

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Abstract
Three children with phenylketonuria between the ages of 2½ and 4½ years have been treated with three different diets low in content of phenylalanine during the past 18 months. In Diets I and III restriction of phenylalanine was accomplished by use of casein hydrolysates from which phenylalanine had been removed and other amino acids added. In Diet II intake of phenylalanine was limited by restriction of total intake of protein in the diet. Diets II and III were commercial preparations. All diets were fed in semisolid form combined with natural foods. After an initial period of hospitalization for regulation of intake of phenylalanine, treatment was carried out at home. Except during the early months of treatment intake of phenylalanine was not severely restricted. Nutritional disturbances were encountered as a result of apparent amino acid deficiencies in Diet I. Inadequate caloric intake and low intake of protein also contributed to poor nutritional status at one stage in the treatment. Biochemical abnormalities characteristic of phenylketonuria were improved when phenylalanine was restricted in the diets of the three children. All three showed improvement in motor ability, increased awareness, lengthened attention span, decreased tenseness and irritability. No change in mental status on objective testing was demonstrated. The improvement in behavior motor control, and ease of handling was such that the parents were unwilling for the children to receive diets with normal content of phenylalanine. This reaction of the parents should be taken into consideration whenever administration of a diet low in content of phenylalanine is considered.

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