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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