Table 5.
Relationship between etiology and radiological findings [occluded vein(s)] in patients with Budd-Chiari syndrome
| Etiology | Anatomical localization of thrombosis at presentation n (%) | ||||||||
| HV only | HV and PV | HV, PV and IVC | HV and IVC | IVC | χ2 | P value | Sig | ||
| PC deficiency | + | 3 (4.3) | 0 (0.0) | 0 (0.0) | 1 (6.3) | 0 (0.0) | 0.001 | 0.99 | NS |
| PS deficiency | + | 1 (1.4) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 0.32 | 0.58 | NS |
| AT III Deficiency | + | 3 (4.3) | 0 (0.0) | 0 (0.0) | 1 (6.3) | 0 (0.0) | 0.001 | 0.99 | NS |
| FVLM | Homo | 9 (17.0) | 0 (0.0) | 1 (12.5) | 0 (0.0) | 9 (17.0) | 0.03 | 0.85 | NS |
| Hetero | 19 (35.8) | 2 (100.0) | 3 (37.5) | 0 (0.0) | 19 (35.8) | ||||
| PGM | Homo | 1 (2.0) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 1 (2.0) | 0.52 | 0.47 | NS |
| Hetero | 2 (4.0) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 2 (4.0) | ||||
| MTHFR | Homo | 7 (14.0) | 0 (0.0) | 1 (14.3) | 0 (0.0) | 7 (14.0) | 0.29 | 0.59 | NS |
| Hetero | 19 (38.0) | 0 (0.0) | 3 (42.9) | 1 (10.0) | 19 (38.0) | ||||
| JAK2 (MPD) | + | 16 (31.4) | 2 (100.0) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 2.08 | 0.15 | NS |
| Primary APA | + | 9 (12.9) | 0 (0.0) | 1 (33.3) | 6 (37.5) | 0 (0.0) | 2.58 | 0.11 | NS |
| Secondary APA | + | 9 (12.9) | 0 (0.0) | 0 (0.0) | 1 (6.3) | 1 (33.3) | 0.02 | 0.88 | NS |
| Behçet’s disease | + | 2 (2.9) | 0 (0.0) | 2 (66.7) | 7 (43.8) | 1 (33.3) | 21.25 | < 0.0001 | HS |
| PNH | + | 2 (2.9) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 0.62 | 0.43 | NS |
Sig: Significance; NS: Not significant; HS: Highly significant; HV: Hepatic vein; PV: Portal vein; IVC: Inferior vena cava; PC: Protein C; PS: Protein S; AT: Antithrombin; FVLM: Factor V Leiden mutation; PGM: Prothrombin gene mutation; MTHFR: Methylene tetrahydrofolate reductase; JAK2: Janus tyrosine kinase-2; MPD: Myeloproliferative disorder; APA: Antiphospholipid antibody syndrome; PNH: Paroxysmal nocturnal hemoglobinuria.