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What is the difference between Gilbert and Crigler-Najjar syndrome?

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Gilbert’s syndrome is an inherited condition often discovered by accident, such as when someone has a blood test. It occurs due to a defect in the processing of bilirubin by the liver. The syndrome may cause the skin and the whites of the eyes to have a yellow tinge due to the build-up of bilirubin.
Gilbert’s syndrome is typically harmless and treatment isn’t required.

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What is the difference between Gilbert and Crigler-Najjar syndrome?

Morbus Gilbert is a mild hyperbilirubinemia, which is only of significance in the case of drug therapy or differential diagnosis. Crigler-Najjar syndrome II leads to a more serious kind of hyperbilirubinemia.

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Gilbert syndrome characterized by jaundice with intermittent elevations of indirect bilirubin, in the absence of hemolysis or underlying liver disease, has both autosomal dominant and recessive inheritance. Crigler-Najjar syndrome type II (CNS2) is a hereditary disorder of bilirubin metabolism characterized by unconjugated hyperbilirubinemia due to reduced and inducible activity of hepatic bilirubin glucuronosyltransferase (GT).

We report 20 children between ages 5 to 15 years with unconjugated hyperbilirubinemia who were given seven days of oral phenobarbitone (5mg/kg/day) and a decrease in the level of bilirubin was noted.


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