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Development of minimally invasive (13)C-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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Detalles Bibliográficos
Autores principales: Turki, Abrar, Stockler, Sylvia, Sirrs, Sandra, Salvarinova, Ramona, Ho, Gloria, Branov, Jennifer, Rosen-Heath, Annie, Bosdet, Taryn, Elango, Rajavel
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9109185/
https://www.ncbi.nlm.nih.gov/pubmed/35585965
http://dx.doi.org/10.1016/j.ymgmr.2022.100880