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KMID : 0367420040470080892
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Journal of Korean Pediatric Society
2004 Volume.47 No. 8 p.892 ~ p.895
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Two Cases of Rotor Syndrome in Siblings.
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Park Hwa-Jin
Kim Eun-Sung
Chung Ji-Young
Cha Sung-Ho
Kim Deog-Yoon
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Abstract
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Rotor syndrome is a rare benign familial disorder characterized by chronic, fluctuating, nonhemolytic and predominantly direct bilirubinemia with normal liver tissue. We have recently experienced two cases of Rotor syndrome in a brother and sister. They revealed icteric sclerae with mild hepatomegaly in physical examination. Laboratory findings showed increased serum bilirubin with direct bilirubin predominance. The urinary excretion of total coproporphyrin was slightly elevated. The 99mTc-DISIDA scan showed a markedly decreased hepatic uptake and poor visualization of gallbladder and biliary tree which could be compatible to the Rotor syndrome. We report two cases with a review of the literature. (Korean J Pediatr 2004;47:892-895)
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KEYWORD
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Rotor syndrome, Hyperbilirubinemia
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