title: [Effect of a purine-free diet on the purine phosphoribosyltransferase activity of erythrocytes in patients with Lesch-Nyhan syndrome].
abstract: Activity of purine phosphoribosyl transferases was studied in 11 patients with Lesch-Nyhan syndrome before and within 1 or 3 months after beginning of the purine-free diet treatment. The enzymatic activity tended to normalization within 1 month after the dietary treatment but the ratio of the enzymes activity was deteriorated within the subsequent 3 months of treatment. A positive clinical effect of the purine-free diet was noted.
mesh_terms: D000228:Adenine Phosphoribosyltransferase / Q000097:blood; D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D004032:Diet; D004912:Erythrocytes / Q000201:enzymology*; D006801:Humans; D007041:Hypoxanthine Phosphoribosyltransferase / Q000097:blood; D007926:Lesch-Nyhan Syndrome / Q000097:blood / Q000178:diet therapy / Q000201:enzymology*; D008297:Male; D010430:Pentosyltransferases / Q000097:blood*; D011687:Purines / Q000008:administration & dosage
chemical_list: D011687:Purines; D010430:Pentosyltransferases; C022484:purine phosphoribosyltransferase; D000228:Adenine Phosphoribosyltransferase; D007041:Hypoxanthine Phosphoribosyltransferase
vernacular_title: Vliianie bespurinovoĭ diety na aktivnost' purinfosforiboziltransferazéritrotsitov u bol'nykh s sindromom Lesha-Nikhana.