We studied the etiology, diagnosis and natural course of myeloproliferative disease (MPD) in 40 consecutive patients with Budd-Chiari syndrome (BCS). In 38% of the BCS patients with MPD another etiological factor was found. JAK2 mutation was present in 41% of the tested BCS patients. Survival was not significantly affected by the presence of MPD.

An 11 year old boy developed pancytopenia, haemolysis, and Budd-Chiari syndrome. The venous thrombosis extended to involve other intra-abdominal vessels before paroxysmal nocturnal haemoglobinuria was recognised as the underlying haematological abnormality. Earlier diagnosis would have made curative bone marrow transplantation a possibility.

BACKGROUND The role of percutaneous hepatic vein angioplasty in the management of Budd-Chiari syndrome has not been well defined. Over a 10 year period at our unit, we have often used this technique in cases of short length hepatic vein stenosis or occlusion, reserving surgical mesocaval shunting for cases of diffuse hepatic vein occlusion or failed angioplasty. AIMS To review the outcome of ...

Among the common hemostatic issues of patients liver cirrhosis, deep venous thrombosis, pulmonary embolism, and intrahepatic thrombus development are more often ignored. With an estimated frequency ranging from 0.6 to 26 percent, portal thrombosis is another potentially dangerous consequence cirrhosis. Objectives: To determine various acquired thrombophilic / hypercoagulable states seen in with...

The Budd-Chiari Syndrome is associated with hepatic venous outflow obstruction. This syndrome is rare in infants and children. As this syndrome is uncommon, especially in the paediatric age group, misdiagnoses and delays in the diagnosis are frequent. A high index of clinical suspicion along with a radiological aid and a histopathological correlation can lead to an early diagnosis and an approp...

Inferior vena cava (IVC) stenting in patients suffering from Budd-Chiari syndrome (BCS) is sometimes complicated by stent migration or misplacement. Here, we describe a novel stent anchoring technique to prevent this complication while using balloon-mounted Palmaz stent for angioplasty of short-segment stenosis in the IVC.

Sir, Mahmoud ef a/. have recently reported underlying myeloproliferative disorders (MPD) as the commonest aetiology in Budd-Chiari syndrome (BCS) in their series. There appears to be continuing controversy over the aetiological factors and their geographical variation observed in this disorder. Earlier studies from Western countries have documented polycythemia vera as the commonest aetiology, ...