title: The effect of ethanol on glucose production in phosphorylase b kinase deficiency.
abstract: Glucose production was measured using stable isotopic techniques in two patients with phosphorylase b kinase deficiency before and after oral ethanol (0.75 g/kg). Glucose production was normal before the ethanol. In one patient, who did not take the full dose of ethanol, glucose production rose initially and then fell. In the other, glucose production fell steadily and in both patients blood lactate concentrations rose. Blood glucose concentrations decreased. Patients with this enzyme deficiency are dependent on the gluconeogenic pathway when fasting and, therefore, ethanol may be potentially hazardous.
authors.affiliation: Department of Child Health, Institute of Child Health, London, UK.
authors.forename: J E
authors.initials: JE
authors.lastname: Collins
authors.forename: K
authors.initials: K
authors.lastname: Bartlett
authors.forename: J V
authors.initials: JV
authors.lastname: Leonard
authors.forename: A
authors.initials: A
authors.lastname: Aynsley-Green
mesh_terms: D000293:Adolescent; D000428:Alcohol Drinking; D001786:Blood Glucose / Q000096:biosynthesis*; D000431:Ethanol / Q000494:pharmacology*; D005943:Gluconeogenesis / Q000187:drug effects*; D006801:Humans; D007700:Kinetics; D007773:Lactates / Q000097:blood; D008297:Male; D010764:Phosphorylase Kinase / Q000172:deficiency*; D010763:Phosphorylase b / Q000172:deficiency*; D006005:Phosphorylases / Q000172:deficiency*
chemical_list: D001786:Blood Glucose; D007773:Lactates; D000431:Ethanol; D010763:Phosphorylase b; D006005:Phosphorylases; D010764:Phosphorylase Kinase