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Intrahepatic Biliary Atresia With Xanthomatosis. Presented by Dr. Lewis Shapiro and Evelyn Richter.
This 5-year-old white boy is the product of a normal full-term pregnancy and uneventful delivery. From the age of 10 days he has had varying degrees of jaundice with scleral icterus, hepatomegaly from 1 to 6 cm, and occasional splenomegaly to 3 cm. Acholic stools and dark urine have been present at times. He bruises easily and has had occasional nosebleeds. With the passage of time there has been a gradual diminution of the above findings.During the past four years, generalized xanthomas have developed; some of these discharge a yellow substance when scratched. The teeth have been green since they first erupted. Flat xanthomata have developed on the oral alveolar mucosa. Generalized pruritus has been a problem for approximately two years. Four grams of cholestyramine resin per day have been given continuously for the past
NEW YORK ACADEMY OF MEDICINE. Arch Dermatol. 1966;94(6):791–797. doi:10.1001/archderm.1966.01600300115024
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