Table 3.
Clinical features of the two groups of adult GSD Ia patients with and without gout
| Variable | Total (n = 95) | Gout group (n = 31) | Controls (n = 64) | P |
|---|---|---|---|---|
| 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 |
| Clinical features | ||||
| Symptoms | ||||
| 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* |
| Hepatic involvement | ||||
| 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 |
| Metabolic complications/comorbidities | ||||
| 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 |
| Laboratory findings | ||||
| SUA, μmol/L | ||||
| 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* |
| LA, mmol/L | ||||
| 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 |
| TG, mmol/L | ||||
| 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 |
| TC, mmol/L | ||||
| 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 |
| Treatment | ||||
| 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 |
Abbreviation: SUA serum uric acid, LA lactic acid, TG triglycerides, TC total cholesterol, LDL-C low-density lipoprotein cholesterol, FBS fasting blood sugar, eGFR estimated glomerular filtration rate, WBC white blood cell, Hgb haemoglobin, PLT platelet, ALT alanine aminotransferase, AST aspartate aminotransferase, GGT gamma-GT, ALP alkaline phosphatase, ULT urate-lowering therapy
*P < 0.05