DEVELOPMENT OF THE COMPONENT COMPOSITION OF AMINO ACID MIXTURES FOR THE NUTRITION OF PATIENTS WITH PHENYLKETONURIA

According to the Ministry of Health in the Republic of Belarus in 2019, there were about 500 patients with phenylketonuria (PKU). Phenylketonuria is a hereditary disease associated with a violation of the metabolism of amino acids, in particular phenylalanine (FA). This disease is accompanied by the accumulation of phenylalanine and its toxic products in the tissues, which leads to severe damage to the central nervous system, manifested in the form of impaired mental development. Many years of world experience shows that for the treatment of such patients, a special diet is prescribed using amino acid mixtures that do not contain phenylalanine or contain it in small amounts, as well as low-protein products based on starch, which are necessary to ensure an adequate energy value of the diet. The article presents the stages of development of the component composition of domestic amino acid mixtures for the nutrition of patients with phenylketonuria, taking into account their age characteristics.

A facile method for urinary phenylalanine measurement on paper-based lab-on-chip for PKU therapy monitoring

A miniaturized paper-based lab-on-chip (LoC) was developed for the facile measurement of urinary Phe (phenylalanine) level on PKU (Phenylketonuria) treated patient.