Pediatric endocrinology

Endogenous Glucose Production in Patients With Glycogen Storage Disease Type Ia Estimated by Oral D-[6,6-2H2]-glucose

J Clin Endocrinol MetabPublished Prospective matched case-control mechanistic study

Study context

Population
Adults with GSDIa and age-, sex- and BMI-matched healthy volunteers (HVs)
Participants
10 vs 10
Comparison
Patients with GSDIa vs matched healthy volunteers
  • GSDIan = 10
  • HVsn = 10reference
Outcome
Rate of appearance of endogenous glucose into the bloodstream (Ra) after a single oral stable-isotope glucose tracer dose
Methodological detailsage-, sex-, and BMI-matched healthy volunteers, stable isotope tracer
  • Age-, sex-, and BMI-matched healthy volunteers
  • Stable isotope tracer

Main finding

As reported in the abstract

Time-averaged glucose Ra
Time-averaged glucose Ra significantly higher in postprandial vs fasted HVs
P < .05
Progressive decrease in glucose Ra in preprandial/fasted GSDIa; change in Ra time-course directly correlated with change in capillary glucose
P < .05

Observational data: associations, not effects.

Parts of this Brief are withheld: the automatic checks could not confirm them against the abstract.

Original publication

Endogenous Glucose Production in Patients With Glycogen Storage Disease Type Ia Estimated by Oral D-[6,6-2H2]-glucose. J Clin Endocrinol Metab. Published 10 September 2023. doi:10.1210/clinem/dgad537

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