Pathologie (Fach) / Leber (Lektion)

Vorderseite Budd-Chiari syndrome
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Etiology:- Secondary to conditions associated with hypercoagulability (most common):→ Polycythemia vera (most important cause) → Paraneoplastic thrombocytosis→ Pregnancy→ Clotting disorders (e.g., factor V Leiden thrombophilia, antiphospholipid syndrome)→ Side effect of medication (e.g., hormonal contraception)- Secondary to invasion or compression of the hepatic veins (less common):→ Hepatocellular carcinoma, renal carcinoma→ Chronic infections and hepatic lesions (e.g., amebiasis, aspergillosis, syphilis, tuberculosis)

Clinical presentation:- Abdominal pain- Tender hepatomegaly- Ascites and abdominal distention- Jaundice- Signs of increased perfusion of portocaval anastomoses

Diagnostics:- Blood analysis: often nonspecific findings; possible elevated aminotransferases- Ascites fluid analysis: WBC count < 500 /μL; Serum ascites albumin gradient > 1.1- Imaging (confirmatory test): Doppler ultrasound; CT, MRI with contrast

Treatment:- Anticoagulation (to prevent propagation of the thrombus)- Restore blood flow: Localized thrombolysis; Balloon angioplasty and stenting- TIPS (transjugular intrahepatic portosystemic shunt) if complications of portal hypertension occur

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