Dietary Management of the Glycogen Storage Diseases: Evolution of Treatment and Ongoing Controversies

Adv Nutr. 2020 Mar 1;11(2):439-446. doi: 10.1093/advances/nmz092.

Abstract

The hepatic glycogen storage diseases (GSDs) are a group of disorders where abnormal storage or release of glycogen leads to potentially life-threatening hypoglycemia and metabolic disturbances. Dietary interventions have markedly improved the outcome for these disorders, from a previously fatal condition to one where people can do well with proper care. This article chronicles the evolution of dietary management and treatment of the hepatic GSDs (types 0, I, III, VI, IX, and XI). We examine historic and current approaches for preventing hypoglycemia associated with GSDs. There is a lack of consensus on the optimal dietary management of GSDs despite decades of research, and the ongoing controversies are discussed.

Keywords: Glycosade; dietary management; glycogen storage disease; hypoglycemia; ketosis; lactate; protein; treatment; uncooked cornstarch.

Publication types

  • Historical Article
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Consensus
  • Diet, Ketogenic
  • Dietary Carbohydrates / administration & dosage
  • Glucosidases / genetics
  • Glycogen / metabolism
  • Glycogen Storage Disease / diet therapy*
  • Glycogen Storage Disease / genetics
  • Glycogen Storage Disease / history
  • History, 20th Century
  • History, 21st Century
  • Humans
  • Hypoglycemia / etiology
  • Hypoglycemia / prevention & control
  • Parenteral Nutrition, Total
  • Portacaval Shunt, Surgical
  • Starch / therapeutic use

Substances

  • Dietary Carbohydrates
  • Starch
  • Glycogen
  • Glucosidases