Liver and intrahepatic bile ducts-nontumor - Unconjugated hyperbilirubinemia

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Liver and intrahepatic bile ducts-nontumor

Jaundice

Unconjugated hyperbilirubinemia

Reviewers: Komal Arora, M.D. (see Reviewers page)
Revised: 15 May 2012, last major update May 2012
Copyright: (c) 2004-2012, PathologyOutlines.com, Inc.

General
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● Due to excessive production, reduced hepatic intake or impaired conjugation
● Excessive production: hemolytic anemia, internal hemorrhage with blood resorption, ineffective hematopoiesis
● Reduced hepatic uptake: Gilbert syndrome (some cases), drug interference with membrane carrier system
● Impaired conjugation: physiologic jaundice of newborn (decreased UGT activity and decreased excretion because hepatic function does not fully mature until 2 weeks old), breast milk jaundice, genetic deficiency of UGT (Crigler-Najjar syndrome types I & II), Gilbert syndrome (some cases), diffuse hepatocellular disease (hepatitis, cirrhosis)
● Crigler-Najjar syndrome type I: autosomal recessive, no UGT activity, fatal in neonates due to kernicterus
● Crigler-Najjar syndrome type II: autosomal dominant, decreased UGT activity, mild kernicterus; yellow skin
● Gilbert syndrome: may be autosomal dominant, UGT activity is 30% of normal due to mutations in promoter region, asymptomatic, affects 6% of US population, detected during periods of stress (illness, strenuous exercise, fasting, Eur J Pediatr 2012;171:11 ); living donor liver transplantation safe for both Gilbert disease donors and their recipients (Exp Clin Transplant 2012;10:39)

End of Liver and intrahepatic bile ducts-nontumor > Jaundice > Unconjugated hyperbilirubinemia

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