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What are the nutritional implications of hereditary phenylketonuria?

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Final answer:

Phenylketonuria (PKU) is a genetic disorder that affects the breakdown of phenylalanine. It can cause damage to the central nervous system and brain, leading to symptoms such as delayed neurological development and intellectual disability. A strict diet low in phenylalanine is necessary to manage the condition.

Step-by-step explanation:

Phenylketonuria (PKU) is a genetic disorder where individuals lack the enzyme phenylalanine hydroxylase, which results in the inability to properly break down phenylalanine. This leads to toxic levels of phenylalanine in the body, causing damage to the central nervous system and brain. It can result in symptoms such as delayed neurological development, intellectual disability, seizures, rash, and tremors. Pregnant women with PKU are at risk of exposing the fetus to excessive phenylalanine, which can lead to complications in fetal development.

People with PKU must follow a strict diet that is low in phenylalanine to avoid symptoms and damage. They should avoid foods high in phenylalanine, such as certain animal products, starches, and artificial sweeteners containing aspartame. Early diagnosis and adherence to a modified diet can help minimize the severity of symptoms.

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