caa a22 4500 469043776 CHVBK 20180323132818.0 cr unu---uuuuu 170328e19920101xx s 000 0 eng 10.1007/BF01800337 doi (NATIONALLICENCE)springer-10.1007/BF01800337 Kirkman Jr H. Brain and Development Research Center and Department of Pediatrics, University of North Carolina School of Medicine at Chapel Hill, 27599-7250, NC, USA aut Erythrocytic uridine diphosphate galactose in galactosaemia [Elektronische Daten] [H. Kirkman Jr] Summary: An earlier claim of a deficiency of uridine diphosphate galactose in erythrocytes of galactosaemia patients was not confirmed. Enzymic techniques similar to those of the earlier investigators were used to determine not only the concentration of uridine diphosphate galactose but also the ratio of this concentration to the sum of the uridine sugar diphosphates (uridine diphosphate galactose and uridine diphosphate glucose). The values in erythrocytes of galactosaemic subjects were similar to those of non-galactosaemic children on a galactose-restricted diet and to those of normal adults. These results cast doubt on the claim of a major deficiency of uridine diphosphate galactose in galactosaemia and on the need for treating galactosaemic children with uridine. SSIEM and Kluwer Academic Publishers, 1992 Journal of Inherited Metabolic Disease Kluwer Academic Publishers 15/1(1992-01-01), 4-16 0141-8955 15:1<4 1992 15 10545 https://doi.org/10.1007/BF01800337 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/BF01800337 text/html Onlinezugriff via DOI NATIONALLICENCE 100 1- Kirkman Jr H. Brain and Development Research Center and Department of Pediatrics, University of North Carolina School of Medicine at Chapel Hill, 27599-7250, NC, USA aut NATIONALLICENCE 773 0- Journal of Inherited Metabolic Disease Kluwer Academic Publishers 15/1(1992-01-01), 4-16 0141-8955 15:1<4 1992 15 10545 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer