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| Author |
정민규 ( Min Kyu Jung ) , 배명환 ( Myung Hwan Bae ) , 김대진 ( Dae Jin Kim ) , 이완석 ( Wan Suk Lee ) , 조창민 ( Chang Min Cho ) , 탁원영 ( Won Young Tak ) , 권영오 ( Young Oh Kweon ) |
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| Title |
고빌리루빈혈증 가족력을 가진 Rotor 증후군 1예 |
| Publicationinfo |
Korean J Gastroenterol 2007 Apr; 049(04): 251-255. |
| Key_word |
Rotor syndrome, Hyperbilirubinemia, Hereditary |
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| 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 bilirubinmia. 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. (Korean J Gastroenterol 2007;49:251-255) |
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