Genetic disorders of bilirubin metabolism

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Disorders of Bilirubin Metabolism

Bilirubin is the end product of degradation of the heme moiety of hemoproteins. Hemoglobin, derived from senescent erythrocytes, is the major source of bilirubin. Significant fractions are also derived from other hemoproteins of liver and other organs. Historically, hyperbilirubinemia has attracted the attention of clinicians as a marker of liver dysfunction. Subsequently, the studies of biliru...

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Bile bilirubin pigment analysis in disorders of bilirubin metabolism in early infancy.

BACKGROUND Early and accurate diagnosis of Crigler-Najjar syndrome, which causes prolonged unconjugated hyperbilirubinaemia in infancy, is important, as orthotopic liver transplantation is the definitive treatment. AIM To determine whether bilirubin pigment analysis of bile in infants with prolonged unconjugated hyperbilirubinaemia provides useful diagnostic information in the first 3 months ...

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Bilirubin metabolism.

DURING the last ten years considerable advances have been made in our knowledge of the metabolism of bilirubin and thus in our understanding of the basic defects underlying jaundice, particularly in the newborn and in some types of familial hyperbilirubinaemia. These advances are due mainly to the characterization of 'direct' bilirubin as an ester glucuronide of bilirubin. Many of the classical...

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Bilirubin metabolism: Applied physiology

ont matter & 2005 2005.10.002 thor. Tel.: +1 718 . howdhur@aecom. Summary Bilirubin is the breakdown product of the haem moiety of haemoglobin and other haemoproteins. Because of internal hydrogen bonding, bilirubin is water-insoluble and requires enzyme-mediated glucuronidation in the liver for biliary excretion. In normal circumstances, plasma bilirubin is mostly unconjugated and is tightly b...

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Bilirubin metabolism in the fetus.

Bilirubin metabolism was studied in dog and monkey fetuses. Bilirubin-(3)H was administered to fetal animals in utero by prolonged intravenous infusion. Fetal plasma disappearance, hepatic uptake, biliary excretion, and placental transfer of bilirubin-(3)H were measured.Bilirubin metabolism and excretion in the fetus was much less efficient than in the adult. Fetal plasma levels of tritium were...

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ژورنال

عنوان ژورنال: World Journal of Gastroenterology

سال: 1998

ISSN: 1007-9327

DOI: 10.3748/wjg.v4.isuppl2.20