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<正> Background: Portal biliopathy producing intrahepa-tic and extrahepatic biliary ductai abnormalities fromportal hypertension, particularly with extrahepaticportal vein obstruction (EHPVO) is common. Amajority of these patients are asymptomatic, but oc-casionally there is symptomatic biliary obstruction,and cholangitis and choledocholithiasis.Objective: To explore the principles of diagnosis andtreatment of portal biliopathy.Data sources: To review the literature of portal bili-opathy.Conclusions: Endoscopic sphincterotomy, stone ex-traction and supportive drainage could effectively re-lieve cholangitis when jaundice is associated withcommon bile duct stones. Definitive decompressiveportal-system vein shunting operation and choledo-cho-jejunostomy are sometimes required when biliaryobstruction is recurrent and progressive.