PSC is a progressive chronic hepatobiliary disorder of unknown etiology for which no effective medical or surgical therapy now exists. This syndrome occurs most commonly in young men and is frequently associated with CUC. The diagnosis is best made utilizing endoscopic retrogradecopic retrograde cholangiography. Although liver histologic findings alone are infrequently diagnostic of PSC, it remains important to exclude other causes of chronic cholestasis. Although the etiology remains unknown, preliminary studies suggest that PSC is related to immunologic damage. Although viral infections can induce obliterative cholangitis in children, their role in the cause of PSC remains undefined. PSC progresses slowly from an asymptomatic stage to cirrhosis, portal hypertension, and premature death secondary to liver failure. Bile duct cancer appears to be a frequent complication of long-standing PSC. Since no therapy is of established efficacy, controlled clinical trials of both medical and surgical therapy should be encouraged. Fat-soluble vitamin deficiencies commonly occur in the advanced stages of PSC, and therefore serum levels of vitamins A, D, E, and prothrombin time should be monitored on a regular basis to prevent complications associated with these deficiencies. Liver transplantation is now a therapeutic option for the treatment of end-stage PSC. Palliative surgical biliary drainage procedures, proctocolectomy, and surgical decompressive shunts that increase the risk of liver transplants, therefore, should be avoided, if possible, in young PSC patients. If a total proctocolectomy is surgically indicated, we would strongly recommend performing a ileoanal pull-through procedure, thus, avoiding the formation of an abdominal ileostoma and the risk of developing bleeding peristomal varices.
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