31st July 2023

Dubin-Johnson Syndrome

Dubin-Johnson syndrome is an autosomal recessive disorder causing a conjugated hyperbilirubinaemia. Typically, patients present in adolescence with intermittent jaundice and right upper quadrant pain. It often worsens during pregnancy.

It is due to a defect in the canalicular multispecific organic anion transporter (cMOAT) protein, leading to defective hepatocyte excretion of conjugated bilirubin. Therefore, unlike the two conditions above, urobilinogen will be raised in the urine. Liver biopsy shows a characteristic pigmented, ‘black’ liver.

No treatment is required.