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Type 1 Tyrosinemia (HT-1)


Type 1 tyrosinemia is an inherited metabolic disease characterized by the inability of the individual affected to metabolize a specific amino acid, tyrosine.

Amino acids are the constituent units of proteins and are therefore daily taken as part of one’s diet. Individuals with type 1 tyrosinemia should follow a diet that is free from tyrosine and another amino acid involved in the disease, phenylalanine, as a precursor to tyrosine.

Given the impossibility of excluding these amino acids from natural proteins, the patient should, in general, follow a protein-free diet, supplemented with adequate protein substitutes (without tyrosine and phenylalanine). The treatment is, however, customized according to the needs of the individual patient.

PIAM, always attentive to the problems related to rare or low-prevalence diseases, offers a diversified range of specific protein substitutes for the correct nutritional management of patients with type 1 tyrosinemia, providing tailor-made solutions for all ages, from infancy to adulthood.