title: [Role of vitamin B6 in treating children with hereditary metabolic pathology].
abstract: Possibilities of vitamin B6 treatment of patients with hereditary pathology of metabolism are discussed. Special attention is paid to the vitamin B6-dependent conditions characterized by elevated pyridoxine requirements. High pyridoxine doses were successfully used for the treatment of patients with hereditary vitamin B6-dependent xanthurenuria under the control of renal excretion of tryptophan metabolites (xanthurenic and kynurenic acids, kynurenin, N1-methylnicotinamide) and 4-pyridoxic acid. Interrelation was traced between the severity of the disease and the necessary pyridoxine doses. Patients with the most pronounced clinical and biochemical changes needed especially high doses of the vitamin (200 mg/day). Use of vitamin B6 in a dose of 100 mg/day in pyridoxine-dependent homocystinuria induced a remission of the biochemical parameters on the 4th day of the treatment. It is noted that the efficacy of the treatment is dependent on the timely starting of the therapy and pyridoxine doses necessary for normalization of the clinical and biochemical parameters in each patient.
mesh_terms: D000799:Angioedema / Q000378:metabolism; D002648:Child; D002675:Child, Preschool; D004485:Eczema / Q000378:metabolism; D005260:Female; D006712:Homocystinuria / Q000378:metabolism; D006801:Humans; D007223:Infant; D008297:Male; D008661:Metabolism, Inborn Errors / Q000188:drug therapy* / Q000378:metabolism; D009450:Neurodermatitis / Q000378:metabolism; D009751:Nutritional Requirements; D011736:Pyridoxine / Q000627:therapeutic use*; D014364:Tryptophan / Q000378:metabolism; D014581:Urticaria / Q000378:metabolism; D026681:Vitamin B 6 Deficiency / Q000378:metabolism; D014977:Xanthurenates / Q000652:urine
chemical_list: D014977:Xanthurenates; D014364:Tryptophan; D011736:Pyridoxine
vernacular_title: Rol' vitamina B6 v lechenii deteĭ s nasledstvennoĭ patologieĭ obmena veshchestv.