The dietary treatment of hepatic glycogen synthetase deficiency.
A child with glycogen synthetase deficiency has been treated for one year by more frequent feeding with a diet lower in carbohydrate and higher in protein than her previous diet. This treatment virtually abolished the overnight hyperketonaemia and daytime hyperglycaemia which occurred before treatment. On the new dietary regime the child has had no clinical symptoms of hypoglycaemia and her growth velocity has increased dramatically.
D004040:Dietary Carbohydrates; D004041:Dietary Fats; D004044:Dietary Proteins; D005260:Female; D006008:Glycogen Storage Disease / Q000178:diet therapy*; D006006:Glycogen Synthase / Q000172:deficiency*; D006801:Humans; D006943:Hyperglycemia / Q000178:diet therapy; D007231:Infant, Newborn; D007657:Ketone Bodies / Q000097:blood; D008099:Liver / Q000201:enzymology*
D004040:Dietary Carbohydrates; D004041:Dietary Fats; D004044:Dietary Proteins; D007657:Ketone Bodies; D006006:Glycogen Synthase