An 8-month-old with phenylketonuria (PKU) is to be discharged, and the nurse is writing discharge instructions. According to those instructions, which foods should be avoided?
Explanation
• Children with PKU must avoid foods high in phenylalanine, which include milk, soy, eggs, meat, fish, beans, cheese, chocolate, and regular grains.
• PKU is an autosomal recessive metabolic disorder. The absence of the liver enzyme phenylalanine hydroxylase prevents the conversion of phenylalanine, an essential amino acid, into tyrosine. As a result, excessive phenylalanine builds up in the bloodstream and tissues, causing permanent damage to brain tissue.
• The parents must calculate the total intake of phenylalanine in foods they feed their child that contain a moderate amount of phenylalanine. The foods that are allowed but must be moderated include bananas, potatoes, PKU milk, concentrated fruit juices, and ice cream.
• Foods that can be eaten without calculation of phenylalanine include most fruits, lettuce, tomatoes, carrots, butter and margarine, and low-protein grains.