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Rotor Syndrome

  • Rotor Syndrome is the result of a hereditary mutation causing defective transport of conjugated bilirubin across the hepatocyte membrane and into the bile canaliculi. Because the actual enzymatic process of bilirubin conjugation is not affected (See: Heme Degradation), bilirubin is conjugated normally in the hepatocytes but cannot be properly excreted due to the transport defect. Consequently, a conjugated hyperbilirubinemia ensues resulting signs of jaundice. As can be seen, the basic pathogenesis of Rotor Syndrome is very similar to that of Dubin-Johnson Syndrome; however, there are some mild biochemical differences in metabolism between the two disorders and there is no accumulation of pigment in the liver of patients with Rotor Syndrome.