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

ABSTRACT:

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 ut

SUBMITTER: Turki A 

PROVIDER: S-EPMC9109185 | biostudies-literature | 2022 Jun

REPOSITORIES: biostudies-literature