الفهرس 
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Abstract
B
CS is not a primary disease of the liver parenchyma but subsequent liver dysfunction following obstruction of hepatic veins or the suprahepatic Inferior Vena Cava, hepatic venous outflow obstruction results in an elevated sinusoidal pressure and leads to hepatic congestion.
Usually, congestion is followed by subsequent centrilobular fibrosis and nodular regenerative hyperplasia that lead to chronic liver dysfunction and cirrhosis; in some instances, however, it results in fulminant hepatic failure requiring emergency liver transplantation.
BCS is a life-threatening group of disorders resulting from hepatic venous outflow obstruction, that may occur at the level of the hepatic venules (hepatic veno-occlusive disease), the large hepatic veins, inferior vena cava (IVC), or the right atrium (congestive hepatopathy).
The clinical presentation is highly variable; from being asymptomatic to fulminate, acute, sub-acute, and chronic subtypes depending on duration of the disease, biochemical disturbance, and liver histology.
FVL (rs6025) is a variant of human factor V which causes an increase in blood clotting (hypercoagulability). Due to this mutation, Protein C, an anticoagulant protein which normally inhibits the pro-clotting activity of factor V, is not able to bind normally to Factor V, leading to hypercoagulable state.
The role of the G20210A mutation of the prothrombin gene appears to be negligible in BCS patients. Other recently identified inherited risk factors have not been extensively studied in BCS patients.
The prevalence of C677T MTHFR polymorphism appears to be increased in BCS patients worldwide, but a causal association has not been clearly established in European patients.
This study was carried out on 35 newly of Budd Chiari syndrome patients who were attending tropical department at Ain Shams University Hospitals during the period from April 2020 to December 2020. Informed consent was obtained from all patients in the study.
In the current study, Factor V Leiden Mutation was detected in 65.7%of patients, followed by MTHFR mutation which was detected in 48.6% of patients.
Similar to our study, Factor V Leiden mutation (FVLM) was the most common etiological cause of BCS (53.1%) as reported in the Egyptian BCS patients followed by mutation of the gene encoding methylene tetrahydrofolate reductase (MTHFR) (51.6%)