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Table 2 Clinical features of the two subgroups of adult GSD Ia-related gout patients with and without gouty tophi

From: Clinical features of gout in adult patients with type Ia glycogen storage disease: a single-centre retrospective study and a review of literature

Variable

Patients with gouty tophi (n = 15)

Patients without gouty tophi (n = 16)

P

Male

11 (73.3)

9 (56.3)

0.320

Family history of HUA

1 (6.7)

1 (6.3)

0.962

Age, years

28.1 ± 6.6

23.4 ± 5.4

0.036*

Age at GSD diagnosis, years

13 (2,39)

11 (3,30)

0.147

Gout symptom onset age, years

18 (10,29)

19 (13,28)

0.383

Gout symptom duration, years

6.1 ± 5.4

3.8 ± 2.1

0.042*

Number of involved joints

4.6 ± 1.5

2.4 ± 1.8

0.001*

Upper extremity involved

12 (80.0)

3 (18.8)

0.014*

Renal calculi

11 (73.3)

4 (25.0)

0.005*

Haematuria

5 (33.3)

3 (18.8)

0.354

Proteinuria

5 (33.3)

8 (50.0)

0.347

Renal insufficiency

1 (6.7)

1 (6.3)

0.962

eGFR

119.1 ± 53.4

141.8 ± 46.7

0.218

Kidney enlargement

5 (33.3)

6 (37.5)

0.901

Anaemia

10 (66.7)

10 (62.5)

0.809

Hepatic adenoma

12 (80.0)

8 (50.0)

0.081

SUA, umol/L

812.1 ± 126.9

789.7 ± 137.9

0.641

TG, mmol/L

10.8 ± 6.9

13.8 ± 13.0

0.430

TC, mmol/L

6.3 ± 2.3

7.8 ± 3.2

0.146

LDL-C, mmol/L

3.0 ± 0.7

3.5 ± 1.5

0.268

LA, mmol/L

9.4 ± 4.3

8.9 ± 3.1

0.722

FBS, mmol/L

2.9 ± 0.7

2.9 ± 0.7

0.909

Height, cm

159.2 ± 6.1

159.5 ± 10.2

0.932

BMI, kg/m2

21.2 ± 4.0

21.5 ± 4.3

0.894

  1. Values are expressed as numbers (%), median (range) or mean ± standard deviation
  2. Abbreviation: HUA hyperuricaemia, GSD Ia glycogen storage disease type Ia, eGFR estimated glomerular filtration rate, SUA serum uric acid, TG triglycerides, TC total cholesterol, LDL-C low-density lipoprotein cholesterol, LA lactic acid, FBS fasting blood sugar, BMI body mass index
  3. *P < 0.05