Endogenous Glucose Production in Patients With Glycogen Storage Disease Type Ia

Study design: An investigator-initiated human pilot-study. Study population: Ten adult subjects with GSDIa and ten age and gender-matched healthy subjects. Interventions: Three experiments will be performed for each subject. During the first in hospital experiment, two oral D-\[6,6-2H2\]glucose loads will be performed 2 hours before breakfast and at lunchtime, respectively. The third oral D-\[6,6-2H2\]glucose load will be performed at home (random time). Capillary blood samples will be collected on filter paper at specific time points after each oral D-\[6,6-2H2\]glucose load. During the experiments, subjects will be monitored by subcutaneous continuous glucose monitoring (CGM).