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Vascular Disorders of the Liver

. Budd-Chiari SyndromeVeno-occlusive DiseaseIschemic HepatitisCongestive HepatopathyPeliosis Hepatitis. Budd-Chiari Syndrome (BCS). Obstruction of hepatic venous outflow- IVC, H. veins, H. venules. Causes: thrombosis or membranous websAcute, subacute, or chronicDx: doppler US or angiogram.

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Vascular Disorders of the Liver

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    1. Vascular Disorders of the Liver

    2. Budd-Chiari Syndrome Veno-occlusive Disease Ischemic Hepatitis Congestive Hepatopathy Peliosis Hepatitis

    3. Budd-Chiari Syndrome (BCS) Obstruction of hepatic venous outflow- IVC, H. veins, H. venules. Causes: thrombosis or membranous webs Acute, subacute, or chronic Dx: doppler US or angiogram. Liver bx: centrilobular congestion, necrosis, and fibrosis.

    4. Common Causes THROMBOSIS (prominent in Western nations): Myeloproliferative d/o’s such as polycythemia vera. Hypercoaguable states such as Factor V Leiden. Infections such as abscess, TB, Schistosomiasis. Cancer: mostly Hepatocellular or Renal. Oral contraceptives or Pregnancy. MEMBRANOUS WEBS (more prominent in India, Africa, and the Far East).

    5. Clinical Features Acute- severe RUQ pain, HM (enlarged caudate lobe), n/v, ascites. Transaminases may be > 1000. Can be fulminant with coagulopathy and encephalopathy. Subacute- Weeks of vague RUQ discomfort, HM, SM, graudual ascites. Chronic- Insidious onset, may be asymptomatic until presenting with portal hypertension.

    6. Treatment Options Anticoagulation, thrombolytic therapy, portalcaval shunt, balloon angioplasty, stents.

    7. Veno-occlusive Disease (VOD) Occlusion of the terminal hepatic venules and hepatic sinusoids. Most often due to toxic injury with radiation and chemotherapy- especially Bone Marrow Transplantation. Rare with herbal teas. Typically occurs 2-4 weeks after BMT. Begins with weight gain and jaundice. Similar to BCS with portal HTN. Dx via liver bx: occluded hepatic venules due to endothelial toxicity.

    8. Ischemic Hepatitis Often called shock liver, follows circulatory failure. Seen in setting of hypotension from CV dz or sepsis. Transaminases often 2-4,000 and LDH as high as 10,000. Peak at day 2 and rapidely trend down. Associated with prolonged PT. Bx: centrilobular necrosis with preserved hepatic architecture.

    9. Congestive Hepatopathy Caused by R Heart failure. Can lead to Cardiac Cirrhosis. RUQ discomfort, varied lft abnormalities, PT usually prolonged. Bx: atrophy of hepatocytes, distended sinusoids, fibrosis.

    10. Peliosis Hepatitis Multiple blood filled cysts in the liver. Associated with Renal transplantation and AIDS. Can be caused by Bartonella henselae (catch-scratch dz organism).

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