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Nutrition Library: Special Considerations


Phenylketonuria (PKU) results from reduced activity of the enzyme that converts phenylalanine (PHE), an amino acid, to tyrosine, another amino acid. Due to this enzyme deficiency, an increased concentration of PHE builds up in the blood, and insufficient amounts of tyrosine are made. Severe brain damage leading to mental retardation will occur unless dietary treatment is begun within the first three weeks of life.

Individuals with PKU require a diet containing limited phenylalanine and supplemented with tyrosine. Foods such as meat, fish, eggs, cheese, milk products, legumes, and bread, which are high in PHE are eliminated or greatly reduced.

Artificial protein substitutes are given which contain amino acids without phenylalanine. These formulas are designed to meet the specific needs of individuals with PKU.

Measuring and mixing the formula accurately are both very important. For infants under four months, you can use fresh boiled water (cooled) and sterile bottles. For older children, you can mix the formula with water. You can enhance the taste with vanilla, sugar or flavor extracts if your child prefers.

Individuals cannot survive without any phenylalanine. A small amount is required for protein synthesis and normal growth. Your health care provider will give you a very specific amount of phenylalanine to adhere to in yours or your dchild's diet.

It is recommended to distribute the phenylalanine exchanges evenly throughout the day.

Sugar substitutes are made with phenylalanine and aspartic acid. Thus, diet drinks and other products with these ingredients should be avoided.

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