Abstract

SELECTED CASE A year after being told that she had abnormal liver test results, a 52-year-old white woman presented with increasing fatigue, pruritus, diffuse abdominal pain, and jaundice. Although endoscopic retrograde cholangiopancreatography was indicated, a diagnostic laparotomy was undertaken. An intraoperative cholangiogram revealed beading and narrowing of intrahepatic and extrahepatic bile ducts characteristic of primary sclerosing cholangitis. Cholecystectomy, exploration of the common bile duct, and endoscopic dilation of large-duct biliary strictures were performed. During the ensuing 12 months the pruritus became severe. Her entire body itched; she slept poorly and felt like she was on a cactus bed. She scratched until she bled, but scratching did not relieve the pruritus. Conventional doses and schedules of cholestyramine, hydroxyzine hydrochloride, and ursodeoxycholic acid had failed to provide her with relief and her pruritus was considered to be intractable. When referred to the National Institutes of Health, she was scratching continuously and was

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