Supplemental files from Endogenous glucose production in patients with glycogen storage disease type Ia estimated by oral D [6,6-2H2]-glucose

 Supplemental file 1. Calculated M₂ curve in study participants during glucose-SIB test 1 (blue),  glucose-SIB test 2 (red) and glucose-SIB test 3 (green). Less than 25% decrease in  concentration between the peak and the last time point were observed in subject 008 (glucose-SIB  test 1), subject 019 (glucose-SIB test 2) and subject 020 (glucose-SIB test 2) disqualifying these data  for computational analysis.

 Supplemental file 2. Capillary glucose concentrations in study participants during glucose-SIB test 1 (A) and 2 (B).

  

Supplemental file 3. Calculated model parameters in the study participants. Data in italics were excluded from calculations. (A) GSDIa patients with attenuated phenotype. (B) GSDIa patients with a severe phenotype. (C) Healthy volunteers.