A patient with hereditary hemorrhagic telangiectasia (HHT) may develop acute hepatic distintegration with hepatic failure. This probably occurs as a result of marked vascular shunting within the liver.


Features of acute hepatic disintegration in HHT:

(1) The patient develops widespread ischemia within the liver causing biliary and hepatocellular necrosis.

(2) The episode may be triggered by an infection, pregnancy or other cause.

(3) There is the sudden onset of severe right upper quadrant pain followed by progressive liver dysfunction.

(4) A liver biopsy shows disruption of the normal hepatic architecture with hepatocellular necrosis, bile duct necrosis, extravasation of bile and hemorrhage.


Differential diagnosis: fulminant hepatitis from other causes


Liver transplantation may be necessary if the patient progresses to hepatic failure..


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