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Table 1 Genetic and biochemical profile of the GSD Ia patients enrolled in study (n = 11, baseline)

From: A triple-blinded crossover study to evaluate the short-term safety of sweet manioc starch for the treatment of glycogen storage disease type Ia

ID Genotype Gender (M/F) Age (years) Comorbidities Body weight (Kg) Height (cm) BMI (kg/m2) Starch-dosage 4/4 h (cm) Glucose (mmol/L) Lactic acid (mmol/L) Insulin (UI/mL) Uric acid (mg/dL) TC(mg/dL) HDL (mg/dL) TG (mg/dL)
A c.[247C>T];[820G>A] F 24 Adenomas, nephrolithiasis 72.1 160 28.2 45 5.3 2.55 29.1 7.7 324 25 1997
B c.[113A>T];[1039C>T] F 16 76.8 163 28.9 47 4.0 1.69 4.7 6.2 198 44 307
C c. [247C>T];[247C>T] F 27 Hepatomegaly, hepatic hypervascular nodule 64.2 149 28.9 60 5.6 2.48 8.9 6.8 217 36 384
D c.[113A>T];[1039C>T] F 16 71.9 166 26.1 61 6.1 1.74 30.8 7.8 156 23 344
E c.[247C>T];[247 C>T] M 23 Nephrolithiasis,  hepatic steatosis 78.6 164 29.2 65 6.2 1.79 34.0 6.9 170 22 383
F c.[247C>T];[809G>T] F 21 Adenomas 59.0 153 25.2 68 5.3 1.38 16.7 6.5 144 35 122
G c.[247C>T];[247C>T] M 16 Nephrolithiasis 82.0 169 28.7 73 4.1 3.10 5.7 8.2 196 21 484
H c.[189G>C];[1039C>T] M 20 78.0 176 25.2 77 5.6 1.65 22.3 8.5 246 39 746
I c.[1039C>T];[1039C>T] M 22 109.5 170 37.9 78 5.9 1.93 20.3 7.8 244 34 448
J c.[113A>T];[323C>T] M 27 72.0 167 25.8 85 5.1 2.10 14.9 6.8 338 37 608
K c.[247C>T];[563-3C>G] M 26 Mild auditive loss 75.2 170 26.0 90 5.7 3.51 43.2 10.0 242 30 553
  1. M: male, F: female. Reference values: glucose (> 3.8 mmol/L); lactate (0.5–2.2 mmol/L); insulin (1.4 -14 µUI/mL); total cholesterol (TC, < 200 mg/dL); HDL cholesterol (> 35 mg/dL); triglycerides (TG, < 150 mg/dL) and uric acid (3.4 -7 mg/dL). In bold, abnormal values