Patient's question:
My little sister has phenylketonuria and has been undergoing treatment since she was a child. Her condition is currently stable, but now she wants to have children and is already pregnant. It's urgent to consult about what foods would be beneficial for both her and her child.What kind of help is needed: What foods can people with phenylketonuria eat?
Doctor's answer:
Phenylketonuria (PKU) is a hereditary metabolic disorder caused by a deficiency of enzymes in the phenylalanine metabolic pathway, which prevents phenylalanine from being converted into tyrosine, leading to the excretion of large amounts of phenylalanine and other metabolites in the urine of affected infants. Currently, there are no specific drugs available, and the main approach is to prevent the recurrence of complications through dietary control. For infants, specially formulated low-phenylalanine formula can be provided, and during the weaning period, complementary foods should be based on low-protein foods such as starches, vegetables, and fruits. When necessary, multiple medications should be used for treatment and control. Treatment can include oral tetrahydrobiopterin (BH4) and levodopa. In addition to low-phenylalanine ingredients, a diet rich in fruits and vegetables is recommended.