Bilirubin Metabolism and Jaundice

Allan W. Wolkoff, Paul D. Berk

Research output: Chapter in Book/Report/Conference proceedingChapter

4 Scopus citations

Abstract

Bilirubin, a degradation product of heme, circulates bound to albumin, from which it is extracted in the liver. It is translocated from blood to bile by a multistep process involving hepatocellular uptake, intracellular binding, conjugation with glucuronic acid, and active transport into bile. Hyperbilirubinemias are predominantly unconjugated, mainly conjugated, or mixed. Those with normal common liver function tests are often familial and benign; abnormalities of other hepatic tests suggest a condition with potentially serious sequelae. Studies of familial hyperbilirubinemias have identified specific proteins and pathways essential for normal liver function, including bilirubin transport and metabolism. Mutations in particular proteins underlie four of the five familial hyperbilirubinemias: the Gilbert, Crigler-Najjar types 1 and 2, and Dubin-Johnson syndromes. The basis for Rotor syndrome remains unknown. Many different mutations in three protein components of the canalicular bile secretory apparatus are likewise the basis for several familial cholestasis syndromes.

Original languageEnglish (US)
Title of host publicationSchiff's Diseases of the Liver
PublisherWiley-Blackwell
Pages120-151
Number of pages32
ISBN (Print)0470654686, 9780470654682
DOIs
StatePublished - Oct 31 2011

Keywords

  • Benign recurrent intrahepatic cholestasis
  • Bilirubin
  • Bilirubin conjugation
  • Crigler-Najjar syndrome(s)
  • Dubin-Johnson syndrome
  • Gilbert syndrome
  • Hyperbilirubinemia
  • Intrahepatic cholestasis of pregnancy
  • Jaundice
  • Progressive familial intrahepatic cholestasis
  • Rotor syndrome

ASJC Scopus subject areas

  • Medicine(all)

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  • Cite this

    Wolkoff, A. W., & Berk, P. D. (2011). Bilirubin Metabolism and Jaundice. In Schiff's Diseases of the Liver (pp. 120-151). Wiley-Blackwell. https://doi.org/10.1002/9781119950509.ch6