Phenylketonuria (PKU), monitoring of Phenylalanine
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Phenylketonuria (PKU) is a rare inherited disease that prevents
the essential amino acid phenylalanine from being properly metabolized.
(An essential amino acid is required for normal growth, development,
and body functioning and must be obtained from the diet, as the
body cannot make it.) Because of this, phenylalanine can accumulate
in the body and cause health problems including mental retardation.
In the U.S. and many other countries, routine screening for PKU
is required for all newborns. In the U.S., about 1 in 15,000 babies
is born with PKU. People with PKU are placed on a special diet with
a severe restriction of phenylalanine from birth to adolescence
or after. Women with PKU must remain on the special diet throughout
pregnancy. Since individuals with PKU must consider aspartame as
an additional source of phenylalanine, aspartame-containing foods
must state "Phenylketonurics: Contains Phenylalanine" in the U.S.
