Table 2.
Parameter | WD (n=55) | Non-WD (n=158) | P † | ||
---|---|---|---|---|---|
|
|
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Median (IQR) | n (%)* | Median (IQR) | n (%)* | ||
Demographics | |||||
| |||||
Age (yr) | 34 (20 to 43) | - | 36 (20 to 51) | - | 0.160 |
| |||||
Male gender | - | 33 (60.0) | - | 96 (60.8) | 0.921 |
| |||||
Clinical presentation | |||||
| |||||
Hepatic | - | 22 (40.0) | - | 65 (41.1) | <0.001 |
| |||||
Neurologic | - | 17 (30.9) | - | 68 (43.0) | |
| |||||
Neuropsychiatric | - | 7 (12.7) | - | 20 (12.7) | |
| |||||
Combination | - | 9 (16.4) | - | 0 (0.0) | |
| |||||
None, e.g., donors, family screening | - | 0 (0.0) | - | 5 (3.2) | |
| |||||
Biochemical feature | |||||
| |||||
Caeruloplasmin (g/L) | 0.04 (0.03 to 0.07) | - | 0.25 (0.22 to 0.29) | - | <0.001 |
| |||||
Total copper (µmol/L) | 2.70 (1.26 to 4.85) | - | 15.91 (12.80 to 18.60) | - | <0.001 |
| |||||
NCC1 (µmol/L) | 0.19 (−0.34 to 2.09) | - | 3.94 (2.51 to 6.15) | - | <0.001 |
| |||||
NCC2 (mcg/L) | 7.10 (−26.75 to 117.62) | - | 204.17 (119.80 to 337.45) | - | <0.001 |
| |||||
NCC% (%) | 12 (−20 to 39) | - | 26 (19 to 33) | - | 0.012 |
| |||||
CCR (µmol/g) | 7.04 (5.19 to 10.19) | - | 8.39 (7.62 to 9.24) | - | 0.012 |
| |||||
Adjusted copper (µmol/L) | 17.12 (16.54 to 17.83) | - | 17.51 (15.97 to 18.96) | - | 0.697 |
| |||||
Discriminant function | −2.35 (−2.55 to −1.94) | - | 0.79 (0.37 to 1.44) | - | <0.001 |
*Percentage calculated based on total within group. †Medians based on Mann-Whitney U test and frequencies based on Pearson’s chi-square test. Value is significant at P<0.05. CCR: copper-caeruloplasmin ratio, IQR: interquartile range, NCC%: non-caeruloplasmin copper percentage, NCC1: non-caeruloplasmin copper in molar unit, NCC2: non-caeruloplasmin copper in mass unit