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skos:definition |
MSH: A benign, autosomally recessive inherited hyperbilirubinemia characterized by the presence of a dark pigment in the centrilobular region of the liver cells. There is a functional defect in biliary excretion of bilirubin, cholephilic dyes, and porphyrins. Affected persons may be asymptomatic or have vague constitutional or gastrointestinal symptoms. The liver may be slightly enlarged, and oral and intravenous cholangiography fails to visualize the biliary tract.,NCI: An inherited autosomal recessive disorder characterized by the presence of mild jaundice due to abnormalities in the bilirubin transportation from the liver parenchyma to the biliary system.
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