Table 2.
Clinical features of the two subgroups of adult GSD Ia-related gout patients with and without gouty tophi
| 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 |
Values are expressed as numbers (%), median (range) or mean ± standard deviation
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
*P < 0.05