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PKU is an inherited disorder of phenylalanine metabolism, caused by a deficiency of the enzyme phenylalanine hydroxylase, which leads to mental retardation if untreated. The aim of dietary management is to prevent the accumulation of phenylalanine by means of a low phenylalanine diet. Protein requirements are met by supplementing the diet with a phenylalanine free amino acid mixture. Phenylalanine requirements are met by including a small amount of natural protein in the diet In certain circumstances (such as pregnancy) an additional tyrosine supplement may be required.

Phenylketonuria is an inherited disorder that affects about 1 in every 10,000 people: they cannot break down the protein from food in the usual way. Protein is made up of a number of amino acids, one of which is called phenylalanine. We all need protein to help the body grow, repair and function.

Phenylalanine is also involved in brain growth and sending nerve signals around the body.  Sufferers of this metabolic disorder do not produce the chemical needed to break down phenylalanine. As a result the level of phenylalanine builds up in their blood.  In a baby these high levels would result in brain damage.

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