vascular disorders of the liver Flashcards

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1
Q

what are the vascular diseases of the liver divided into ?

A

disorders of portal venous inflow
disorders of hepatic venous outflow

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2
Q

what is budd chiari ?

A

obstruction of one or more major hepatic veins and is characterized by hepatomegaly, ascites and abdominal pain
this includes the IVC and smaller hepatic venules

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3
Q

which myeloproliferative disorder is associated with budd chiari ?

A

polycythemia vera

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4
Q

what are the clinical features of the acute form of budd chiari ?

A

abdominal pain
nausea and vomiting
tender hepatomegaly
ascites

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5
Q

what are the clinical features of the chronic form of budd chiari ?

A

enlargement of the liver
mild jaundice
sudden onset ascites
negative hepatojugular reflux
splenomegaly with portal hypertension

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6
Q

what is the most common cause of budd chiari ?

A

hepatic venous thrombosis

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7
Q

what are the risk factors of budd chiari ?

A

any hypercoagulable state and thrombosis

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8
Q

why is there caudate lobe hypertrophy ?

A

because the caudate lobe drains directly into the inferior vena cava and is not affected by hepatic venous thrombosis

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9
Q

when is liver transplantation indicated in budd-chiari syndrome ?

A

in chronic budd chiari and in the fulimant form

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10
Q

what investigatiion is mmandatorry in budd-chiari ?

A

thrombophilia screening

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11
Q

what are the features of the ascitic fluid associated with budd-chiari?

A

high total protein content
elevated albumin levels

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12
Q

what is the management for acute BCS ?

A

anticoagulants and thrombolytic therapy can be given
ascites should be treated with diuretics
treat underlying cause
TIPS
Angioplasty

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13
Q

what must be screened for in chronic BCS ?

A

hepatocellular carcinoma

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14
Q

what is the lifelong management after TIPS and transplantation ?

A

life long anti coagulants

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15
Q

what are the risk factor for portal venous thrombosis ?

A

local factors and general factors
local factors : malignant tumors within the venous territory , cirrhosis
general factors : inherited or acquired hypercoaguable states

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16
Q

other name for sinusoidal obstructive syndrome ?

A

hepatic veno-occlusive disease

17
Q

when does SOS happen ?

A

after high dose chemotherapy (cyclophosphamide) and jamaican drinkers of bush tea , first 20 to 30 days after bone marrow transplantation

18
Q

what is the clinical presentation of SOS ?

A

resembles budd-chiari - high bilirubin, hepatomegaly, ascites weight gain