Rotor syndrome is an inheritable disorder of bilirubin metabolism characterized by a chronic, benign, nonhemolytic jaundice, due to raised, predominantly direct bilirubin. It is considered a pathophysiologic entity primarily involving the storage of organic anions leading to increased reflux of bilirubin from the hepatocytes back to the plasma, resulting in decreased net hepatic uptake. This concept is further supported by scintigraphic findings(i.e., prolonged visualization of the cardiac blood pool with concomitant delayed and markedly decreased uptake of the radionuclide by the liver cells). Furthermore, this scintigraphic pattern differentiates Rotor syndrome from Dubin-Johnson syndrome, extrahepatic biliary obstruction, or advanced hepatocellular disease.
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