Table 1.
Baseline characteristics of the patients at presentation
| Patient ID | 1 | 2 | 3 | 4 | 5 | 6 | 7 |
| Age at diagnosis (yr) | 34 | 21 | 30 | 21 | 31 | 24 | 25 |
| Symptoms at presentation | Ascites | Oesophageal variceal haemorrhage, abdominal pain | Abdominal pain; ascites | Abdominal pain, ascites | Abdominal pain, fever, mouth ulcers | Ascites, renal failure and sepsis (ITU admission) | Abdominal pain |
| Risk factors for BCS | JAK 2 positive MPD; OCP | JAK 2 positive mutation | None identified | Factor V Leiden; OCP | JAK2 positive MPD (Essential Thrombocythaemia); Factor V Leiden | JAK 2 positive mutation | Factor V Leiden |
| Encephalopathy | None | None | None | None | None | None | None |
| Ascites | Moderate | Mild | Mild | Mild | None initially | Severe | Moderate |
| INR | 1.7 | 1.4 | 1.2 | 1.3 | 1.7 | 1.4 | 1.5 |
| Albumin (g/L) | 28 | 37 | 49 | 49 | 49 | 25 | 26 |
| Bilirubin (umol/L) | 19 | 18 | 20 | 18 | 11 | 51 | 32 |
| ALT (U/L) | - | 31 | - | 57 | - | - | - |
| AST (U/L) | 134 | 49 | 20 | 34 | 27 | 277 | 43 |
| Urea (mmol/L) | 2.7 | 2.3 | 2.9 | 4.7 | 2.9 | 4.4 | 2 |
| Creatinine (mmol/L) | 72 | 43 | 70 | 68 | 51 | 92 | 70 |
| Sodium (mmol/L) | 143 | 137 | 143 | 142 | 140 | 130 | 133 |
| MELD | 19 | 14 | 6 | 10 | 12.37 | 14 | 17 |
| UKELD | 53 | 53 | 48 | 49 | 49 | 49 | 55 |
| Hb (g/L) | 137 | 121 | 155 | 128 | 150 | 147 | 88 |
| WCC (109/L) | 7.9 | 9.6 | 10.9 | 5.7 | 5.7 | 28.8 | 6.8 |
| Platelets (109/L) | 345 | 183 | 307 | 247 | 411 | 400 | 226 |
| Rotterdam PI | 1.116 | 0.072 | 1.12 | 0.07 | 1.08 | 1.244 | 1.168 |
| Clichy PI | 4.39 | 1.99 | 3.13 | 4.04 | 3.44 | 7.54 | 7.55 |
| Liver biopsy | Not done | Not done | Not done | Suggestive of hepatic vein obstruction | Consistent with Hepatic venous outflow obstruction | Not done | Not done |
| Level of obstruction | Left hepatic vein | Hepatic vein | Hepatic vein | Hepatic Vein | Right Hepatic Vein | Left Hepatic vein | Hepatic vein |
| Radiological intervention | TIPSS | TIPSS | None | Angioplasty and Stenting to Hepatic vein | Right Hepatic Vein dilatation | TIPSS | TIPSS |
| Type of TIPSS | Viatorr (covered) | Viatorr (covered) | - | - | - | Memotherm, then Viatorr | Memotherm (Uncovered) |
| Medications post intervention | Warfarin | Warfarin | N/A | Warfarin | Warfarin | Warfarin, Interferon | Warfarin |
| Duration of follow up (yr) | 4 | 5 | 7 | 3 | 13 | 14 | 14 |
| Comments/ complications following intervention | TIPSS Stent redilatation after a week of insertion | TIPSS stent stenosis - needed to be re-dilated in 2 yr | Maintained on oral anticoagulation (warfarin) and did not require any intervention | Vascular Wallstent was re-canalized after 2 yr | Inferior RHV dilated 5 yr after the diagnosis (developed ascites and had compliance issues). | Bleeding from hepatic nodule (with INR > 9). Managed conservatively. Later stent was changed to a covered one for TIPSS stenosis | - |
MPD: Myeloproliferative disorder; TIPSS: Trans-jugular intrahepatic posto-systemic shunt; OCP: Oral contraceptive pills; INR: International normalised ratio; ALT: Alanine aminotransferase; AST: Aspartate aminotransferase; MELD: Model for end-stage liver disease; UKELD: United Kingdom model for end-stage liver disease; Hb: Haemoglobin; WCC: White cell count.