COMBINED THERAPEUTIC NUTRITIONAL PRODUCT FOR THERAPY OF TK2-ASSOCIATED MITOCHONDRIAL MYOPATHY Russian patent published in 2024 - IPC A61K31/7068 A61K31/7064 A61K31/513 A61K47/40 A61P43/00 

FIELD: medicine; pharmaceuticals; food industry.

SUBSTANCE: invention relates to a combined therapeutic nutrition product for the treatment of TK2-associated mitochondrial myopathy. A combined therapeutic nutritional product for the treatment of TK2-associated mitochondrial myopathy with two separate formulations including thymidine and deoxycytidine, respectively, where composition 1 contains the following, in mg:

Thymidine 200–400.0 Microcrystalline cellulose 72.0–144.0 Maltodextrin 69.0–138.0 Hyprolose low-substituted 20.0–40.0 Crospovidone 24.0–48.0 Sodium saccharinate 6.0–12.0 Silicon dioxide 6.0–12.0 Sodium stearyl fumarate 4.0–8.0 Magnesium stearate 4.0–8.0

composition 2 contains the following, in mg:

Deoxycytidine 200–400.0 Microcrystalline cellulose 72.0–144.0 Maltodextrin 69.0–138.0 Hyprolose low-substituted 20.0–40.0 Crospovidone 24.0–48.0 Sodium saccharinate 6.0–12.0 Silicon dioxide 6.0–12.0 Sodium stearyl fumarate 4.0–8.0 Magnesium stearate 4.0–8.0

EFFECT: above-described use of thymidine and deoxycytidine with maltodextrin as a combined therapeutic nutritional product is effective for the treatment of TK2-associated mitochondrial myopathy, ensures the absence of adverse events and undesirable reactions, such as deviations from the norm in biochemical parameters of the liver and kidneys; no diarrhea; the presence of statistically significant differences in the average score of the HFMSE scale, reflecting gross motor skills; statistically significant differences in the average score of all indicators of the HINE-2 Scale; statistically significant differences in the average score of the MRC Scale, which reflects muscle strength; differences in the average need for respiratory support (NIV) before and after use of the product, compared with the use of deoxynucleosides in the form of chemicals; the product also has good organoleptic properties and a pleasant taste.

1 cl, 2 dwg, 15 tbl, 2 ex