특징적인 DISIDA scan 소견을 보인 Rotor 증후군 1예

A 15-year-old boy with Rotor syndrome was studied with 99mTc-DISIDA cholescintigraphy. Rotor syndrome is characterized by chronic, benign, nonhemolytic hyperbilirubinemia, and needs to be differentiated from Dubin-Johnson syndrome. Differentiation between the two hereditary disorders was classically made using histologic findings of hepatic biopsy, bromosulfonphtalein (BSP) curve test, and urinary coproporphyrin isomer excretion. However, liver biopsy is not easily accepted because there is no therapeutic gain. The BSP test is not also advised because of possible fatal anaphylactoid reaction to BSP and urinary coproporphyrin is unavailable because of the limited institutes. Recently, there have been a few reports that describe characteristic cholescintigraphic findings in Rotor syndrome, which can differentiate Rotor syndrome from Dubin-Johnson syndrome. We report a case of Rotor syndrome that showed characteristic cholescintigraphic findings. (Kor J Gastroenterol 2000;36:547 - 551)