Development of minimally invasive 13C-glucose breath test to examine different exogenous carbohydrate sources in patients with glycogen storage disease type Ia

Background: Glycogen storage disease type Ia (GSD Ia) is an autosomal recessive disorder caused by deficiency of glucose-6-phosphatase (G6Pase), resulting in fasting hypoglycemia. Dietary treatment with provision of uncooked cornstarch (UCCS) or a novel modified cornstarch (Glycosade®) is available...

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Detalhes bibliográficos
Publicado no:Molecular Genetics and Metabolism Reports
Main Authors: Abrar Turki, Sylvia Stockler, Sandra Sirrs, Ramona Salvarinova, Gloria Ho, Jennifer Branov, Annie Rosen-Heath, Taryn Bosdet, Rajavel Elango
Formato: Artigo
Publicado em: Elsevier 2022-06-01
Assuntos:
Glycogen storage disease type Ia
Glucose-6-phosphatase
Uncooked cornstarch
Glycosade®
13C-glucose
Breath test
Acesso em linha:http://www.sciencedirect.com/science/article/pii/S2214426922000404
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