Table 3 Clinical features of the two groups of adult GSD Ia patients with and without gout

Male

57 (60.0)

20 (64.5)

37 (57.8)

0.532

Age, years

22 (18, 41)

25 (19, 41)

21.5 (18, 30)

0.098

Age at GSD onset, years

2 (0.1, 15)

2 (0.5, 15)

1.3 (0.1, 9)

0.01*

Age at GSD diagnosis, years

11 (1, 39)

12 (2, 39)

11 (1, 23)

0.047*

Mean time from the onset to diagnosis, years of GSD Ia

9 (0.5, 37)

8 (1, 37)

9 (0.5, 23)

0.391

Family history of GSD

22 (23.2)

6 (19.4)

16 (25.0)

0.541

Family history of HUA

2 (2.1)

2 (6.5)

0 (0)

0.104

  Protuberant abdomen

73 (76.8)

22 (71.0)

51 (79.7)

  Recurrent epistaxis

75 (78.9)

20 (64.5)

55 (85.9)

0.016*

  Growth retardation

70 (73.7)

21 (67.7)

49 (76.6)

0.360

  Hypoglycaemia symptoms

52 (54.7)

13 (41.9)

39 (60.9)

0.081

  Diarrhoea

45 (47.4)

8 (25.8)

37 (57.8)

0.003*

  Hepatomegaly

95 (100)

31 (100)

64 (100)

1.000

  Abnormal liver function

95 (100)

31 (100)

64 (100)

1.000

  Hepatic adenoma

46 (48.4)

20 (64.5)

26 (40.6)

0.029*

  Hepatocellular carcinoma

1 (1.1)

1 (3.2)

0 (0)

0.326

 Splenomegaly

21 (22.1)

8 (25.8)

13 (20.3)

0.545

 Abnormal haematological finding

  Anaemia

49 (51.6)

20 (64.5)

29 (45.3)

0.079

  Elevated platelet count

62 (65.3)

20 (64.5)

42 (65.6)

0.915

  Elevated white blood cell count

15 (15.8)

6 (19.4)

9 (14.1)

0.507

 Renal complication

  Renal calculi

31 (32.6)

15 (48.4)

16 (25.0)

0.023*

  Kidney enlargement

33 (34.7)

11 (35.5)

22 (34.4)

0.915

  Proteinuria

33 (34.7)

13 (41.9)

20 (31.3)

0.305

  Haematuria

17 (17.9)

8 (25.8)

9 (14.1)

0.161

  Renal insufficiency

4 (4.2)

2 (6.5)

2 (3.1)

0.449

  Hyperuricaemia

95 (100)

31 (100)

64 (100)

1.000

  Hyperlipaemia

95 (100)

31 (100)

64 (100)

1.000

  Hyperlacticaemia

95 (100)

31 (100)

64 (100)

1.000

  Fasting hypoglycaemia

95 (100)

31 (100)

64 (100)

1.000

  BMI > 24 (kg/m²)

8 (8.4)

5 (16.1)

3 (4.7)

0.060

  Diabetes mellitus

2 (2.1)

1 (3.2)

1 (1.6)

0.596

 Left atrial enlargement

6 (6.3)

2 (6.5)

4 (6.3)

0.970

 Pulmonary hypertension

3 (3.2)

1 (3.2)

2 (3.1)

0.979

 Atherosclerosis

3 (3.2)

1 (3.2)

2 (3.1)

0.979

 Anterior/middle cerebral artery stenosis

2 (2.1)

1 (3.2)

1 (1.6)

0.596

 Osteoporosis

32 (33.7)

13 (41.9)

19 (29.7)

0.236

  Maximum value

700.4 ± 143.2

800.5 ± 131.0

648.6 ± 120.7

< 0.001*

  Average value

542.8 ± 94.5

606.0 ± 109.6

525.0 ± 82.4

0.001*

  Maximum value

10.2 ± 3.7

9.2 ± 3.7

10.7 ± 3.6

0.052

  Average value

8.2 ± 2.8

7.2 ± 2.1

8.6 ± 2.9

0.055

  Maximum value

12.6 ± 8.8

12.4 ± 10.5

12.7 ± 7.9

0.874

  Average value

8.0 ± 4.1

7.4 ± 4.2

8.2 ± 4.1

0.456

  Maximum value

7.0 ± 2.7

7.1 ± 2.9

7.0 ± 2.6

0.789

  Average value

6.1 ± 2.5

7.3 ± 4.1

5.8 ± 1.7

0.033*

 LDL-C, mmol/L

3.0 ± 1.1

3.3 ± 1.2

2.9 ± 1.1

0.119

 Minimum FBS, mmol/L

2.8 ± 0.6

2.9 ± 0.7

2.7 ± 0.6

0.332

 eGFR, mL/(min.1.73 m²)

134.6 ± 50.3

130.8 ± 50.6

136.5 ± 50.5

0.612

  WBC, × 10⁹/L

7.9 ± 3.1

8.1 ± 3.1

7.7 ± 3.1

0.544

 Hgb, g/L

103.4 ± 20.3

98.2 ± 24.3

106.0 ± 17.5

0.081

 PLT, × 10⁹/L

399.4 ± 152.3

444.5 ± 198.4

376.1 ± 117.2

0.041*

 ALT, μ/L

118.5 ± 75.4

105.8 ± 62.6

125.1 ± 80.9

0.250

 AST μ/L

141.4 ± 99.7

117.4 ± 95.6

153.8 ± 100.2

0.099

 GGT μ/L

155.6 ± 116.1

156.3 ± 140.4

155.3 ± 102.7

0.971

 ALP μ/L

227.7 ± 135.2

226.9 ± 100.4

228.1 ± 50.5

0.969

 Initial age of regular, years of treatment with raw corn starch

13.1 ± 6.9

16.1 ± 9.1

11.7 ± 9.1

0.003*

 Proportion of ULT initiated during asymptomatic HUA stage

47 (49.5)

7 (22.6)

40 (62.5)

< 0.001*

 Age of ULT initiated, years

16.3 ± 3.7

16.6 ± 1.8

16.2 ± 3.9

0.103

 Proportion of treatment with lipid-lowering drugs [n (%)]

63 (66.3)

19 (61.3)

44 (68.8)

0.471