Nutritional therapy for glycogen storage diseases.
Glycogen storage diseases (GSDs) are a group of inherited disorders characterized by enzyme defects that affect the glycogen synthesis and degradation cycle, classified according to the enzyme deficiency and the affected tissue. The understanding of GSD has increased in recent decades, and nutritional management of some GSDs has allowed better control of hypoglycemia and metabolic complications. However, growth failure and liver, renal, and other complications are frequent problems in the long-term outcome. Hypoglycemia is the main biochemical consequence of GSD type I and some of the other GSDs. The basis of dietary therapy is nutritional manipulation to prevent hypoglycemia and improve metabolic dysfunction, with the use of continuous nocturnal intragastric feeding or cornstarch therapy at night and foods rich in starches with low concentrations of galactose and fructose during the day and to prevent hypoglycemia during the night.
Heller
Solange
S
Department of Gastroenterology and Nutrition, Hospital Infantil de México Federico Gómez, México DF, México. solangeheller@hotmail.com
Worona
Liliana
L
Consuelo
Alejandra
A
D002648:Child; D002675:Child, Preschool; D004044:Dietary Proteins / Q000008:administration & dosage / Q000627:therapeutic use; D004750:Enteral Nutrition; D006008:Glycogen Storage Disease / Q000145:classification / Q000178:diet therapy* / Q000628:therapy; D006006:Glycogen Synthase / Q000172:deficiency* / Q000235:genetics; D006801:Humans; D007003:Hypoglycemia / Q000517:prevention & control; D007223:Infant; D007231:Infant, Newborn; D010764:Phosphorylase Kinase / Q000172:deficiency* / Q000235:genetics; D013213:Starch / Q000008:administration & dosage / Q000627:therapeutic use; D016896:Treatment Outcome
D004044:Dietary Proteins; D013213:Starch; D006006:Glycogen Synthase; D010764:Phosphorylase Kinase