Hyperbilirubinemia is a common manifestation in internal medicine. It is divided to conjugated and unconjugated hyperbilirubinemia . Conjugated hyperbilirubinemia usually results from hepatocellular or cholestatic liver disease or from extrahepatic biliary obstruction. Isolated conjugated hyperbilirubinemia is the primary manifestation of two heritable disorders, Rotor and Dublin – Johnson syndromes, and can also be seen in some patients with the syndrome of recurrent benign intrahepatic cholestasis.
Dubin – Johnson syndrome is a rare, benign autosmally inheritd hyperbilirubinemia characterized by presence of dark pigment in the centrilobular regin of the liver cells.
Patients with Dubin – Johnson syndrome may be asymptomatic or have vague constitational or gasterointestinal syndromes. Pruritus is absent, and serum bile acid levels are normal.
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