How is phenylketonuria typically treated?

Phenylketonuria (PKU) is a metabolic disorder characterized by an inability to properly metabolize phenylalanine, an amino acid found in many protein-rich foods. Since elevated levels of phenylalanine can lead to neurotoxicity and significant developmental issues, the cornerstone of treatment is the strict avoidance of phenylalanine in the diet. This involves adhering to a low-phenylalanine diet, which means limiting or eliminating the intake of high-protein foods such as meat, dairy, nuts, and certain grains, as well as using specially formulated low-protein products.

While there are other potential treatments that may complement dietary management, such as tyrosine supplementation, only the avoidance of phenylalanine directly addresses the underlying issue in PKU—high levels of phenylalanine in the body—which is why it is the primary and most effective approach to managing this condition.