Supplemental files

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.

补充文件1. 本研究受试者在第1次葡萄糖-SIB试验（glucose-SIB test，蓝色曲线）、第2次葡萄糖-SIB试验（红色曲线）及第3次葡萄糖-SIB试验（绿色曲线）中的经计算得到的M2曲线。受试者008（第1次葡萄糖-SIB试验）、019（第2次葡萄糖-SIB试验）及020（第2次葡萄糖-SIB试验）的峰值浓度与末次观测时间点的浓度降幅不足25%，因此上述数据不符合计算分析的纳入标准，予以排除。
补充文件2. 本研究受试者在第1次（A）及第2次（B）葡萄糖-SIB试验中的毛细血管血糖浓度。
补充文件3. 本研究受试者的经计算得到的模型参数。其中斜体标注的数据已被排除于本次计算流程之外。(A) 减毒表型糖原贮积症Ia型（Glycogen Storage Disease Type Ia）患者；(B) 重型表型糖原贮积症Ia型患者；(C) 健康志愿者。