In recent years, scientific progress in the field of clinical and molecular genetics, biochemistry has made it possible to isolate from a previously undifferentiated state an extensive group of hereditary diseases associated with metabolic disorders. One of the leading methods of pathogenetic therapy of these severe conditions is dietary treatment. In accordance with modern requirements for quality and safety of medicinal products, a complete line of domestic specialized Nutrigen products has been developed for children and adults suffering from rare hereditary diseases (histidinemia, type 1 glutaric aciduria, homocystinuria, isovalerian acidemia, leucinosis or disease " maple syrup, methyl malonic and propionic acidemia, tyrosinemia) associated with impaired amino acid metabolism. Specialized products of the Nutrigen line take into account the nutritional needs of the amino acids of patients of all the above NBAO of all ages (including women in the preconceptual period and during pregnancy), which opens up broad opportunities for the implementation of adequate diet therapy of patients with NBAO.