Abstract

Introduction and aim: The Budd-Chiari Syndrome (BCS) is redefined as hepatic vein outflow tract obstruction with a very low incidence. We aim to analyze the etiology and clinical character of BCS in Hebei area of North China.Material and methods: The diagnosis of BCS and alcoholic related liver cirrhosis (Alcohol-LC) are according to the guidelines of American Association for the Study of Liver Diseases (AASLD), while the diagnosis of hepatitis B virus related liver cirrhosis (HBV-LC) is according to the guidelines of European Association for the Study of the Liver (EASL). BCS patients including inferior vena cava block (IVC), hepatic vein block (HV) and inferior vena cava combining with hepatic vein block (IVC/HV) are involved in this analysis.Results: The subtype’s distributions of this disease are more frequent for IVC patients compared with HV and IVC/ HV patients. The subsequent analysis shows that the incidence of BCS is more predisposed to Alcohol-LC than HBV-LC (p < 0.001).Conclusion: BCS seem to be associated with Alcohol-LC compared with that of HBV-LC.

Highlights

  • Introduction and aimThe Budd-Chiari Syndrome (BCS) is redefined as hepatic vein outflow tract obstruction with a very low incidence

  • In Western countries, hepatic vein block (HV) block is a more common subtype with clear etiology such as oral contraceptives, pregnancy, myeloproliferative disorders (MPD), paroxysmal nocturnal haemoglobinuria (PNH), and Behcet diseases (BD) [3], while inferior vena cava block (IVC) or IVC/HV combined block has almost no hypercoagulability of blood, which seems to be more common in Asia, and the cause of the disease is interpreted as congenital abnormal development, thrombosis and inflammation [6,7,8,9]

  • The incidence of subtypes for IVC, HV and IVC/HV; B: The distribution frequency for clinical characteristics among three BCS subtypes; C: The laboratory data including transaminase, total bilirubin, prothrombin time (PT) and activation of partial thrombin time (APTT) was compared among these three BCS subtypes

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Summary

Introduction

The Budd-Chiari Syndrome (BCS) is redefined as hepatic vein outflow tract obstruction with a very low incidence. BCS patients including inferior vena cava block (IVC), hepatic vein block (HV) and inferior vena cava combining with hepatic vein block (IVC/HV) are involved in this analysis. The subsequent analysis shows that the incidence of BCS is more predisposed to Alcohol-LC than HBV-LC (p < 0.001). Budd-Chiari syndrome (BCS) was redefined as obstruction of hepatic vein outflow tract. BCS is a rare disease in Western countries; the incidence is relatively high in some Asian regions [4]. According to the location of obstruction, the disease is usually divided into three types: hepatic veins block (HV type), inferior vena cava block (IVC type), and IVC/ HV combination block (IVC/HV type) [2,5]. In Western countries, HV block is a more common subtype with clear etiology such as oral contraceptives, pregnancy, myeloproliferative disorders (MPD), paroxysmal nocturnal haemoglobinuria (PNH), and Behcet diseases (BD) [3], while IVC or IVC/HV combined block has almost no hypercoagulability of blood, which seems to be more common in Asia, and the cause of the disease is interpreted as congenital abnormal development, thrombosis and inflammation [6,7,8,9]

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