The dietary management consists of a diet low in Tyrosine and its precursor Phenylalanine (i.e. low in native protein) with addition of amino acids supplements free from these amino acids.
Tyrosinemia is an inherited rare disorder in metabolism of the amino acid Tyrosine. There are several types of Tyrosinemia, caused by different enzyme defects. If not treated, this condition leads to an accumulation of Tyrosine and Phenylalanine, as well as to formation of toxic metabolites in all body fluids. Depending on the type of the disorder, this can result in severe irreversible physical damages and finally death.