|
Age at BCS diagnosis, median (range)
|
33 (22–45)
|
38 (16–77)
|
0.086
|
|
Male gender, n (%)
|
11/14 (79%)
|
32/92 (35%)
|
0.003
|
|
Geographic origin
| | |
0.0002
|
|
Europe
|
6 (43%)
|
66 (73%)
| |
|
North Africa
|
8 (57%)
|
12 (13%)
| |
|
Type of outflow obstruction
| | | |
|
IVC occlusion, n (%)
|
10/14 (71%)
|
16/91 (18%)
|
< 0.0001
|
|
HV occlusion alone, n (%)
|
4/11 (36%)
|
75/91 (82%)
|
0.002
|
|
Combined HV and IVC occlusion, n (%)
|
6/11 (55%)
|
15/91 (16%)
|
0.009
|
|
Number of hepatic veins thrombosed, median ± SD
|
2
|
3
|
0.017
|
|
1 HV thrombosed, n (%)
|
3/10 (30%)
|
5/91 (5%)
| |
|
2 HV thrombosed, n (%)
|
1/10 (10%)
|
13/91 (14%)
| |
|
3 HV thrombosed, n (%)
|
5/10 (50%)
|
72/91 (79%)
| |
|
Clinical features at baselinea, n (%)
| | | |
|
Ascites
|
6/13 (46%)
|
72/92 (78%)
|
0.036
|
|
Hepatomegaly
|
6/13 (50%)
|
55/92 (60%)
|
0.38
|
|
Splenomegaly
|
2/12 (17%)
|
51/92 (55%)
|
0.014
|
|
Abdominal pain
|
6/13 (46%)
|
62/92 (67%)
|
0.21
|
|
Hepatic encephalopathy
|
0/13
|
7/90 (8%)
|
0.59
|
|
Gastrointestinal bleeding
|
0/13
|
10/92 (11%)
|
0.36
|
|
Laboratory at baselinea
| | | |
|
ALT < 5 × ULN, n (%)
|
10/11 (91%)
|
60/92 (65%)
|
0.10
|
|
AST < 5 × ULN, n (%)
|
10/11 (91%)
|
63/92 (68%)
|
0.17
|
|
Bilirubinemia (μmol/l), med(min-max)
|
13 (6–38)
|
31 (7 – 207)
|
< 0.0001
|
|
Albuminemia (g/l), med(min-max)
|
37(26–49)
|
35(19–52)
|
0.94
|
|
CRP level (mg/L), med(min-max)
|
85(7–238)
|
16(3–344)
|
0.003
|
|
Creatinemia (μmol/L), med(min-max)
|
80(44–120)
|
73(36–428)
|
0.34
|
|
Prothrombin Time < 70%, n (%)
|
5/11 (45.5%)
|
62/92 (67.4)
|
0.19
|
|
Thrombopenia (< 150.10 [9]/L), n (%)
|
2/12 (16.7%)
|
18/92 (19.6%)
|
1
|
|
Rotterdam BCS index
|
1 (0.0;1.2)
|
1.2 (0.0;3.9)
|
0.0007
|
