Pediatrics Question #10
What statement(s) by the nurse would be appropriate after reviewing anticipatory guidance with
parents of an infant diagnosed with phenylketonuria? (Select all that apply.)
- “A diet low in phenylalanine is necessary.”
- “Exclude meat and dairy from your child’s diet.”
- “Tyrosine should not be included in your child’s diet.”
- “You will need to use a special infant formula.”
The correct answers are A, B, and D. The infant with phenylketonuria (PKU) (a rare metabolic disorder) cannot break down any foods that contain phenylalanine (an amino acid). Phenylalanine is found in many foods with protein such as meat, fish, cheese, nuts, beans, and meat. These foods must be excluded from the infant’s diet. Regular infant formula and breast milk contain phenylalanine. A specialized infant formula that is phenylalanine-free will be used. Phenylalanine normally is metabolized into tyrosine but with PKU the amino acid builds up in the bloodstream.