| Autor: |
Abrar Turki, Sylvia Stockler, Sandra Sirrs, Ramona Salvarinova, Gloria Ho, Jennifer Branov, Annie Rosen-Heath, Taryn Bosdet, Rajavel Elango |
| Jazyk: |
angličtina |
| Rok vydání: |
2022 |
| Předmět: |
|
| Zdroj: |
Molecular Genetics and Metabolism Reports, Vol 31, Iss , Pp 100880- (2022) |
| Druh dokumentu: |
article |
| ISSN: |
2214-4269 |
| DOI: |
10.1016/j.ymgmr.2022.100880 |
| Popis: |
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 to treat hypoglycemia, yet choice of carbohydrate to achieve a desirable glycemic control is debated.13C-glucose breath test (13C-GBT) can be used to examine glucose metabolism from different carbohydrate sources via 13CO2 in breath. Objectives: Our objectives were: 1) establishing the use of a minimally invasive 13C-GBT to examine in vivo glucose metabolism in healthy adults, and 2) using 13C-GBT to measure utilization of the standard UCCS vs. Glycosade® in GSD Ia and healthy controls. Design: Experiment 1- Ten healthy adults (6F: 4 M, 22-33y) underwent 13C-GBT protocol twice as a proof-of-principle, once with oral isotope dose (glucose 75 g + [U-13C6] d-glucose 75 mg) and once without isotope (only glucose 75 g) to test sensitivity of natural 13C-enrichment. Breath samples were collected at baseline and every 20 min for 240 min. Rate of CO2 production was measured at 120 min using indirect calorimetry. Finger-prick blood glucose was measured using a glucometer hourly to test hypoglycemia (glucose |
| Databáze: |
Directory of Open Access Journals |
| Externí odkaz: |
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