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Phenylalanine & Phenylketonuria (PKU) |
Phenylalanine is an essential amino acid. Our bodies cannot make phenylalanine; it must be obtained from our diet. It is found in all protein-containing foods including cheese, eggs, meat, fish and mothers' milk. It is also a component of aspartame.
Phenylketonuria (PKU) is a rare inherited disease in which individuals cannot properly metabolize the amino acid phenylalanine. In the United States, one in 15,000 babies is born with PKU. An infant with PKU who ingests phenylalanine is at risk for health problems including mental retardation. A PKU screening is required by law as part of every baby's first blood test in the United States.
Early detection of PKU and subsequent treatment with diets low in foods that contain phenylalanine help children with PKU enjoy healthy lives. People with PKU are placed on a phenylalanine-restricted diet from birth until adolescence or later. Women with PKU must remain on the diet throughout pregnancy.
All individuals with PKU must consider aspartame as an additional source of phenylalanine; therefore, products that contain aspartame have a label, which reads 'Contains a source of phenylalanine.'
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