Age and Serum Creatinine Can Differentiate Wilson Disease Patients with Pseudonormal Ceruloplasmin
Table 1
Baseline characteristics of 86 WD patients with different phenotypes tested in the first hospitalization.
Variables
ALL
Hepatic type
HN-type†
Neurologic type
value
Patients (n)
86
51
27
8
Cp ≥ 200 mg/L (n, %)
28 (32.6%)
18 (35.3%)
8 (29.6%)
2 (25.0%)
0.784
Sex (male, %)
42 (48.8%)
27 (52.9%)
11 (40.7%)
4 (50%)
0.590
Age at presentation
18.8 (10.8, 28.3)
14.4 (8.0, 23.0)
22.3 ± 9.3
22.5 ± 11.4
0.091
Age at diagnosis
25.1 ± 12.9
21.7 ± 13.0
28.6 ± 9.5
35.0 ± 14.7
0.005
Duration of onset (years)‡
2.0 (0.5, 9.3)
1.0 (0.4, 6.2)
3.0 (1.0, 11.1)
12.5 ± 10.6
0.010
Duration of follow-up (years)
4.9 (1.8, 7.1)
4.1 (1.5, 6.9)
5.9 (3.6, 7.5)
3.9 ± 2.6
0.242
Range (years)
0–9.8
0–9.8
0–8.2
0–7.0
N
Total records
352
194
126
32
N
Times per patient
4 (2, 6)
3 (2, 5)
5 (2, 7)
4.0 ± 2.3
0.460
K–F ring (+, %)
67 (77.9%)
33 (64.7%)
27 (100%)
7 (87.5%)
0.001
Serum Cu (μmol/L)
8.6 ± 3.6
8.6 ± 3.7
8.8 ± 3.7
8.0 ± 2.2
0.852
Serum Cp (mg/L)
180.9 (158.4, 214.8)
186.1 (163.6, 215.2)
174.2 (162.0, 209.1)
153.3 (146.1, 212.1)
0.200
Sco (IU/L)
0.211 (0.160, 0.322)
0.211 (0.174, 0.345)
0.223 ± 0.104
0.236 ± 0.110
0.524
Basic urinary copper (μg/24 h)
170.7 (113.7, 408.3)
155.0 (108.9–479.8)
176.4 (126.1, 429.7)
214.1 ± 117.6
0.826
Urinary copper with DMPS (μg/24 h)
2094.6 (1335.4, 3673.1)
1850.3 (1267.5, 4012.5)
2234.3 (1678.4, 3626.2)
1916.6 ± 1015.9
0.399
Complete blood count
NE (×109/L)
2.4 (1.5, 3.2)
2.2 (1.6, 2.9)
2.3 ± 1.1
3.5 ± 1.2
0.059
PLT (×109/L)
134.5 (81.3, 233.8)
140.0 (79.0, 268.0)
117.0 ± 52.7
199.4 ± 88.1
0.056
RDW (fL)
45.3 ± 7.0
45.5 ± 7.9
45.7 ± 5.8
42.8 ± 2.7
0.551
ALT (IU/L)
43.0 (24.0, 76.8)
60.0 (32.0, 135.0)
28.0 (21.0, 45.0)
36.3 ± 29.6
≤0.001
AST (IU/L)
42.5 (23.8, 71.0)
58.0 (29.0, 84.0)
29.0 (20.0, 39.0)
44.5 (18.0, 70.8)
0.001
γ-GT (IU/L)
50.5 (27.0, 108.8)
66.5 (29.0, 151.5)
40.0 (27.0, 91.5)
37.0 ± 26.6
0.025
AP (IU/L)
118.0 (82.0, 241.5)
146.0 (87.3, 303.5)
113.8 ± 56.7
88.0 (85.5, 116.8)
0.030
CHE (IU/L)
5088.4 ± 2536.6
5206.0 (2170.0, 8229.0)
5357.5 (3164.3, 5900.5)
7032.5 (4101.0, 7622.5)
0.277
Albumin (g/L)
40.2 (32.1, 43.7)
37.8 ± 8.5
38.7 ± 6.5
41.8 ± 3.4
0.394
Total bilirubin (μmol/L)
17.0 (11.0, 32.0)
22.3 (9.4, 41.3)
17.3 (13.0, 26.3)
14.1 ± 5.6
0.433
Cr (μmol/L)
47.2 ± 18.6
44.3 ± 18.6
46.8 (39.2, 54.1)
57.4 ± 19.6
0.173
CHO (mmol/L)
4.2 ± 1.2
4.1 ± 1.0
3.8 (3.4, 4.4)
4.8 ± 0.7
0.054
PT (s)
13.6 (12.7, 16.7)
14.1 (12.6, 18.9)
13.5 (13.0, 16.4)
12.3 ± 1.0
0.021
INR
1.1 (1.0, 1.4)
1.1 (1.0, 1.5)
1.1 (1.1, 1.4)
1.0 ± 0.1
0.072
Liver cirrhosis (n, %)
57 (66.3%)
30 (58.8%)
27 (100%)
0 (0)
≤0.001
Died (n, %)
6 (7.0%)
3 (5.9%)
3 (11.1%)
0 (0)
0.495
Child–Pugh scores
5 (5, 9)
5 (5, 9)
5 (5, 7)
5 (5, 5)
0.051
Class A (n)
59
31
20
8
0.116
Class B (n)
13
8
5
0
Class C (n)
14
12
2
0
†Hepatic and neurologic presentations; ‡from the time of onset to the first hospitalization. The normal distribution parameters are shown as a mean ± standard deviation or else a median and quantile (25%, 75%). K–F ring, Kayser–Fleischer ring; Cp, ceruloplasmin; Sco, serum ceruloplasmin oxidase; DMPS, dimercaptopropane sulfonate; WBC, white blood cell; PLT, platelet; RDW, red cell distribution width; ALT, alanine aminotransferase; AST, aspartate aminotransferase; γ-GT, γ-glutamyltransferase; AP, alkaline phosphatase; CHE, cholinesterase; Cr, creatinine; CHO, cholesterol; PT, prothrombin time; INR, international normalised ratio.
