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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...
Autores principales: | , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Elsevier
2022
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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 |