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Table 1 Biochemical, imaging, clinical and diet therapy data of the patient

From: The biallelic novel pathogenic variants in AGL gene in a chinese patient with glycogen storage disease type III

 

Before

8 days later

20 days later

Clinical data

   

Age (years)

2

2

2

Height (cm)

85

85

85

Weight (kg)

Abdominal circumference(cm)

Right subcostal liver(cm)

14.2

14.2

14.2

59.5

60.0

59.0

10

10

10

Biochemical data

   

Pre-prandial fasting blood glucose (mmol/L)

4.64

5.67

6.1

Nocturnal blood glucose(mmol/L)

-

1.3

5.6

Morning fasting blood glucose (mmol/L)

2.5

4.1

6.5

Lactate (normal range 0.5–2.22mmol/L)

5.54

-

-

Triglycerides (normal range 0–2.26 mmol/L)

5.45

3.71

2.4

Cholesterol (normal range 0–5.2 mmol/L)

5.50

4.96

5.05

Creatine kinase (normal range 40–200 U/L)

213

141

-

Aspartate transaminase (normal range 13–35 U/L)

1154.6

452

532

Alanine transaminase (normal range 7–40 U/L)

781.6

282

165

γ-glutamyl transpeptidase (normal range 7–45 U/L)

98

101

84

Troponin T (normal range 0–0.0223 ng/mL)

21.0

-

-

Myoglobin (normal range 25–58 ng/mL)

0.01

-

-

Imaging data

   

Abdominal ultrasound

Hepatomegaly

-

Hepatomegaly

Below umbilical level 55 mm

Abdominal computed tomography enhanced scan

Hepatomegaly

No obvious abnormal enhancement

-

-

Liver or muscle biopsy

-

-

-

Electromyography

normal

-

-

Electrocardiogram

normal

-

-

Echocardiography

normal

-

-

Chest computed tomography

normal

-

-

Head computed tomography plain scan

-

-

-

Skeletal system x-rays

-

-

-

Diet therapy

Regular diet

Start uncooked cornstarch treatment 1-2 g/kg/d

Adhere to uncooked cornstarch treatment 1-2 g/kg/d

  1. ‘-‘means no done