Final answer:
PKU is a genetic defect where a deficiency in phenylalanine hydroxylase leads to an inability to metabolize phenylalanine efficiently, causing it to accumulate and potentially result in brain damage. It is true that individuals with PKU need to follow a diet low in phenylalanine to prevent these adverse outcomes. Newborns are routinely screened for the condition to enable early dietary management.
Step-by-step explanation:
The statement you've asked about is true. Phenylketonuria (PKU) is a genetic defect characterized by a deficiency in the enzyme phenylalanine hydroxylase, making individuals with the condition unable to metabolize the amino acid phenylalanine effectively. As a result of the enzyme deficiency, phenylalanine accumulates in the body, leading to toxic levels that can cause brain damage and severe intellectual disability if not managed properly.
Individuals with PKU must adhere to a strict diet low in phenylalanine to prevent these harmful outcomes. This includes avoiding foods high in protein, certain starches, and the artificial sweetener aspartame. If the condition is not managed with a controlled diet, phenylalanine and its metabolites can build up and cause neurological problems such as hyperactivity, seizures, and delayed development, among other symptoms. Newborns in the United States and Canada are routinely screened for PKU, and when the condition is detected early and a modified diet is followed, the severity of symptoms can be significantly reduced. However, it's important to note that PKU is a lifelong condition, requiring ongoing management of dietary phenylalanine intake.