Abstract

Phenylketonuria (PKU) is an inborn error of amino acid metabolism manifested by an inability of the body to convert phenylalanine (PA) to tyrosine. The well-known clinical picture is that of a child, normal at birth, in whom mental retardation, seizures, eczema, and imperfect pigmentation develop later. In recent years, the condition has been treated, especially in pregnancy, with artificial diets low in PA.

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