title: [A case with Rotor syndrome in hyperbilirubinemic family].
abstract: Rotor syndrome is a rare, benign familial disorder characterized by chronic fluctuating, nonhemolytic and predominantly conjugated hyperbilirubinemia with normal hepatic histology. In contrast to Dubin-Johnson syndrome, there is no liver pigmentation in Rotor syndrome. A 36-year-old man was admitted due to asymptomatic persistent jaundice. His siblings had jaundice with direct hyperbilirubinemia. Physical examination revealed icteric sclerae without hepatosplenomegaly. Laboratory findings showed increased serum bilirubin with direct bilirubinemia. Hepatic uptake and storage capacity of indocyanine green was markedly reduced, while excretion into bile was slightly suppressed. Markedly decreased hepatic uptake and poor visualization of the gallbladder and biliary tract were shown in 99mTc-DISIDA scan. Histology of the liver showed mild steatosis without pigmentation. We report a case with the review of literature.
authors.affiliation: Department of Internal Medicine, Kyungpook National University School of Medicine, Daegu, Korea.
authors.forename: Min Kyu
authors.initials: MK
authors.lastname: Jung
authors.forename: Myung Hwan
authors.initials: MH
authors.lastname: Bae
authors.forename: Dae Jin
authors.initials: DJ
authors.lastname: Kim
authors.forename: Wan Suk
authors.initials: WS
authors.lastname: Lee
authors.forename: Chang Min
authors.initials: CM
authors.lastname: Cho
authors.forename: Won Young
authors.initials: WY
authors.lastname: Tak
authors.forename: Young Oh
authors.initials: YO
authors.lastname: Kweon
mesh_terms: D000328:Adult; D004396:Coloring Agents / Q000493:pharmacokinetics; D006801:Humans; D006933:Hyperbilirubinemia, Hereditary / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000235:genetics; D007208:Indocyanine Green / Q000493:pharmacokinetics; D007566:Jaundice, Chronic Idiopathic / Q000175:diagnosis* / Q000000981:diagnostic imaging; D008099:Liver / Q000000981:diagnostic imaging; D008111:Liver Function Tests; D008297:Male; D011877:Radionuclide Imaging; D019275:Radiopharmaceuticals; D019785:Technetium Tc 99m Disofenin
chemical_list: D004396:Coloring Agents; D019275:Radiopharmaceuticals; D007208:Indocyanine Green; D019785:Technetium Tc 99m Disofenin