الفهرس 
ACKNOWLEDGMENTS
PathophysiologyOnly 14 pages are availabe for public view
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Abstract
Budd–Chiari syndrome (BCS) was defined according to EASL guidelines as an obstruction of the hepatic venous outflow tract in absence of right sided heart failure or constrictive pericarditis .Hence, lesions causing BCS could be located in the small or large hepatic veins(HV) or in the suprahepatic part of inferior vena cava (IVC).The estimated incidence of BCS is 1 per million per year.
Budd-Chiari syndrome has fo SUMMARY AND RECOMMENDATION
ur clinical variants; acute, subacute, chronic and fulminant. The chronic form is difficult to distinguish from cirrhosis regardless of the etiology.
It can also be classified according to etiology either as primary or secondary. Primary BCS is often associated with the presence of thrombotic risk factors. While, secondary BCS could be caused by obstruction in hepatic veins outflow by injury after surgery e.g: liver transplantation or liver resection, extrinsic compression as by hepatic hydatid cyst ,polycystic liver disease or liver abscess, and could be caused by tumor invasion (i.e. hepatocellular carcinoma).
The management of BCS requires a multidisciplinary approach in all cases with involvement of hepatology, interventional radiology, haematology, histopathology and liver surgery.
The aim of this study is to describe the socio-demographic feature of Budd-Chiari Syndrome (BCS) and to show the prevalence of factor V leiden (FVL) mutation in upper Egyptian patients with Budd- Chiari Syndrome.
In order to reaching to the aim ; 50 patients were enrolled in this study with the following eligibility criteria: Patients previously diagnosed as Budd-Chiari Syndrome based on clinical picture plus laboratory investigation and Radiological assessment and we exclude Patients with hepatitis C-or B related liver cirrhosis or Patient receiving anticoagulant therapy or drugs that alter the hemostatic function within 2 weeks from sample collection.
In the current study there were 28 males (56%) 22 females (44%) with mean age (32.5). The etiological factor was not identified in 22% of cases (n=11). Isolated protein C deficiency was founded in 26%(n=13 ) of patients and protein S deficiency was founded in 10% (n=5). Factor V Leiden mutation was the etiology in 5 patients (10%). Membranous web as well as antiphospholipid syndrome each of them were the etiology in 8% (n=4). Behςet’s disease was diagnosed in 4% (n=2). Obstruction at the level of hepatic veins was presented in 80%.
In this first study of the characteristics of BCS among Upper Egyptian patients, BCS was found to be common in middle aged males. Most patients presented with liver cirrhosis. Isolated protein C deficiency was the most common identified etiology of BCS patients. Factor V Leiden mutation was founded to be the second most common identified etiology . Hepatic veins obstruction was the main pattern of venous involvement.
Finding a cause may be useful in identifying the right approach to treatment. Many patients come in late stage of disease so early diagnosis lead to appropriate treatment and improve survival rate.