Biomaterial Database

The dietary treatment of hepatic glycogen synthetase deficiency. 1977

A Aynsley-Green, and D H Williamson, and R Gitzelmann

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.

UI	MeSH Term	Description	Entries
D007231	Infant, Newborn	An infant during the first 28 days after birth.	Neonate,Newborns,Infants, Newborn,Neonates,Newborn,Newborn Infant,Newborn Infants
D007657	Ketone Bodies	The metabolic substances ACETONE; 3-HYDROXYBUTYRIC ACID; and acetoacetic acid (ACETOACETATES). They are produced in the liver and kidney during FATTY ACIDS oxidation and used as a source of energy by the heart, muscle and brain.	Acetone Bodies,Bodies, Acetone,Bodies, Ketone
D008099	Liver	A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances.	Livers
D004040	Dietary Carbohydrates	Carbohydrates present in food comprising digestible sugars and starches and indigestible cellulose and other dietary fibers. The former are the major source of energy. The sugars are in beet and cane sugar, fruits, honey, sweet corn, corn syrup, milk and milk products, etc.; the starches are in cereal grains, legumes (FABACEAE), tubers, etc. (From Claudio & Lagua, Nutrition and Diet Therapy Dictionary, 3d ed, p32, p277)	Carbohydrates, Dietary,Carbohydrate, Dietary,Dietary Carbohydrate
D004041	Dietary Fats	Fats present in food, especially in animal products such as meat, meat products, butter, ghee. They are present in lower amounts in nuts, seeds, and avocados.	Fats, Dietary,Dietary Fat,Fat, Dietary
D004044	Dietary Proteins	Proteins obtained from foods. They are the main source of the ESSENTIAL AMINO ACIDS.	Proteins, Dietary,Dietary Protein,Protein, Dietary
D005260	Female		Females
D006006	Glycogen Synthase	An enzyme that catalyzes the transfer of D-glucose from UDPglucose into 1,4-alpha-D-glucosyl chains. EC 2.4.1.11.	Glycogen (Starch) Synthase,Glycogen Synthetase,Glycogen Synthase I,Synthase D,Synthase I,UDP-Glucose Glycogen Glucosyl Transferase,Synthase, Glycogen,Synthetase, Glycogen,UDP Glucose Glycogen Glucosyl Transferase
D006008	Glycogen Storage Disease	A group of inherited metabolic disorders involving the enzymes responsible for the synthesis and degradation of glycogen. In some patients, prominent liver involvement is presented. In others, more generalized storage of glycogen occurs, sometimes with prominent cardiac involvement.	Glycogenosis,Disease, Glycogen Storage,Diseases, Glycogen Storage,Glycogen Storage Diseases,Glycogenoses,Storage Disease, Glycogen,Storage Diseases, Glycogen
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man