Related MeSH Hierarchy (4)
Diseases [C] » Nutritional and Metabolic Diseases [C18] » Metabolic Diseases » Metabolism, Inborn Errors » Hyperbilirubinemia, Hereditary » Jaundice, Chronic Idiopathic
Diseases [C] » Pathological Conditions, Signs and Symptoms [C23] » Pathologic Processes » Disease Attributes » Chronic Disease » Jaundice, Chronic Idiopathic
Diseases [C] » Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16] » Genetic Diseases, Inborn » Metabolism, Inborn Errors » Hyperbilirubinemia, Hereditary » Jaundice, Chronic Idiopathic
Diseases [C] » Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16] » Infant, Newborn, Diseases » Hyperbilirubinemia, Neonatal » Jaundice, Neonatal » Jaundice, Chronic Idiopathic
Description
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. MeSH
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