Introduction of Dietary Therapy

Infant formulas for PKU come in a powdered form and are mixed with water and taken as a substitute for regular infant formula or breast milk. In some clinics, only phenylalanine-free formula is given for a few days so that blood phenylalanine will quickly decrease to an acceptable level. A prescribed amount of breast milk or standard infant formula, however, should be shortly introduced into the diet. Whole protein is needed to meet phenylalanine requirements and prevent phenylalanine deficiency, which will lead to muscle protein catabolism and inadequate weight gain. For formula-fed infants, both standard infant formulas and PKU medical foods are used in prescribed amounts and are bottle fed. Breast-feeding of an infant with PKU is possible and, as with all infants, should be encouraged whenever possible. Mature breast milk contains approximately 46 mg 100ml-1of phenylalanine compared to approximately 59 mg 100 ml-1 in cows' milk protein-based formula and approximately 88 mg 100 ml-1 in soy-based formulas. Therefore, breast-fed infants may initially have slightly lower plasma phenylalanine levels. If a mother chooses to continue breast-feeding, she is advised about the proper ratio of breast milk to PKU medical food to feed her infant. The key to either method is frequent monitoring of blood phe-nylalanine and adjusting the diet based on phenyl-alanine intake, weight gain, and blood levels. Guidelines for the frequency of monitoring were also recommended by the NIH consensus panel (Table 3). The method used for monitoring varies

Table 2 Recommended daily nutrient intakes (ranges) for infants, children, and adults with PKU

Age Nutrient

Table 2 Recommended daily nutrient intakes (ranges) for infants, children, and adults with PKU

Age Nutrient

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