Budd Chiari Syndrome Flashcards
(31 cards)
What is normally found in the Portal vein?
Ammonia rich blood
What is the process that occurs in the liver involving ammonia?
NH₃ + CO₂ → Urea
What is a characteristic of Budd Chiari syndrome?
Thrombus formation in hepatic veins
What develops in the liver due to obstruction in Budd Chiari syndrome?
Congestion
What results from centrilobular necrosis in Budd Chiari syndrome?
Fulminant hepatic failure
What types of manifestations can occur in Budd Chiari syndrome?
Acute or chronic manifestations
What type of cause is Budd Chiari syndrome for Portal hypertension?
Post hepatic cause
What is the definition of Budd Chiari syndrome?
Thrombosis of ≥2 hepatic veins
What drains the caudate lobe in normal conditions?
Inferior vena cava (IVC)
What is seen on USG with Doppler if the thrombus involves the IVC?
Enlargement of the caudate lobe
What is a secondary cause of Budd Chiari syndrome?
Hydatid cyst
What types of tumors can cause Budd Chiari syndrome?
HCC or multiple metastases pressing on hepatic veins
What hematological disorder is associated with Budd Chiari syndrome?
Polycythemia vera (JAK 2 mutation)
What condition explains sluggish circulation in the liver and thrombosis?
Erythrocytosis
What is Paroxysmal nocturnal Hemoglobinuria (P.L.G.A gene defect)?
It is a defect in CD 59 which is present on the platelets and inhibits platelet aggregation. This increases the chances of thrombus formation.
What is Deep vein thrombosis?
A condition where blood clots form in deep veins, often in the legs.
What is Thrombotic diathesis?
A condition characterized by a tendency to form clots due to decreased Protein C, S, and AT III, contributing to a hypercoagulable state.
What is Factor V Leiden mutation?
A mutation that leads to defective binding with protein C, activating the extrinsic system and initiating clot formation.
What effect do postpartum and OCPs have on coagulation?
High estrogen levels lead to a hypercoagulable state.
Which tumors are associated with thrombotic diathesis?
Hepatocellular carcinoma, RCC, and Wilm’s tumor can cause thrombotic diathesis by pressing on the hepatic vein.
What infections can contribute to thrombotic diathesis?
Hydatid cyst infections can contribute to thrombotic diathesis.
What connective tissue disorder is associated with thrombotic diathesis?
Anti-phospholipid antibody syndrome, where Anti B2 glycoprotein antibody stimulates the intrinsic system of clotting.
What is Sarcoidosis?
A condition that may be associated with thrombotic diathesis.
What is Bechet’s disease?
A condition that may be associated with thrombotic diathesis.
