buddchiari
Table of Contents
Budd-Chiari syndrome (BCS)
see also:
Introduction
- Budd-Chiari syndrome is a congestive hepatopathy caused by blockage of hepatic veins which also causes raised portal venous pressure which may lead to portal hypertension, ascites, etc.
- occurs in 1/100,000 of the general population worldwide
- 75% of cases are due to thrombosis of the hepatic vein and these are regarded as being “Primary BCS”
- 25% are caused by external compression of the hepatic vein “Secondary BCS”
- this is different to the related condition of veno-occlusive disease which can occur in bone marrow transplant recipients as a complication of their medications, and also with certain herbs
Aetiology
Primary BCS
- pregnancy
- postpartum
- paroxysmal nocturnal haemoglobinuria - 12% develop BCS
- other pro-thrombotic tendencies eg. thrombophilia
- Behçet's disease
- trauma
Secondary BCS
- congental venous webs
- IVC stenosis
Clinical features
- abnormal liver function tests (LFTs)
- jaundice
- hepatosplenomegaly
- ascites
- and later, hepatic encephalopathy
buddchiari.txt · Last modified: 2021/06/03 07:12 by gary1