Rotor 증후군

Rotors syndrome is a rare hereditary disorder characterized by chronic, nonhemolytic and predominantly conjugated hyprbilirubinemia with normal hepatic histology. It resembles the Dubin-Johnson syndrome, the main difference being the absence of brown pigment in the liver cell. Recent studies dernonstrated that these two syndromes are distinct pathophysiologic entities. Authors traced a 19 year-old male patient who had shown persistent and predominant,ly con- jugated hyperbilirubinemia from birth. Urinary excretion of total coproporphyrin was marked- ly increased. Oral cholecystography visualized the gallbladder, but Tc-DISIDA scan showed markedly decreased hepatic uptake and poor visualization of the gallbladder and biliary tree. Histology of the liver was normal. So we confirmed the diagnosis of Rotors syndrome. (Kore- an J Gastroenterol 1994; 26: 592 595)