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KMID : 0614620070490040251
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Korean Journal of Gastroenterology
2007 Volume.49 No. 4 p.251 ~ p.255
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A Case with Rotor Syndrome in Hyperbilirubinemic Family
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Jung Min-Kyu
Bae Myung-Hwan
Kim Dae-Jin
Lee Wan-Suk
Cho Chang-Min
Tak Won-Young
Kweon Young-Oh
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Abstract
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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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KEYWORD
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Rotor syndrome, Hyperbilirubinemia, Hereditary
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