Dietary Management of the Glycogen Storage Diseases: Evolution of Treatment and Ongoing Controversies.
Consensus
Diet, Ketogenic
Dietary Carbohydrates
/ administration & dosage
Glucosidases
/ genetics
Glycogen
/ metabolism
Glycogen Storage Disease
/ diet therapy
History, 20th Century
History, 21st Century
Humans
Hypoglycemia
/ etiology
Parenteral Nutrition, Total
Portacaval Shunt, Surgical
Starch
/ therapeutic use
Glycosade
dietary management
glycogen storage disease
hypoglycemia
ketosis
lactate
protein
treatment
uncooked cornstarch
Journal
Advances in nutrition (Bethesda, Md.)
ISSN: 2156-5376
Titre abrégé: Adv Nutr
Pays: United States
ID NLM: 101540874
Informations de publication
Date de publication:
01 03 2020
01 03 2020
Historique:
received:
06
03
2019
revised:
29
05
2019
accepted:
02
08
2019
pubmed:
31
10
2019
medline:
8
1
2021
entrez:
31
10
2019
Statut:
ppublish
Résumé
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.
Identifiants
pubmed: 31665208
pii: S2161-8313(22)00267-8
doi: 10.1093/advances/nmz092
pmc: PMC7442342
doi:
Substances chimiques
Dietary Carbohydrates
0
Starch
9005-25-8
Glycogen
9005-79-2
Glucosidases
EC 3.2.1.-
Types de publication
Historical Article
Journal Article
Research Support, Non-U.S. Gov't
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
439-446Informations de copyright
Copyright © American Society for Nutrition 2019.
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