Volume 47(8); August

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Korean Journal of Pediatrics 2004;47(8):892-895.
Published online August 15, 2004.
Two Cases of Rotor Syndrome in Siblings
Hwa-Jin Park1, Eun-Sung Kim1, Ji-Young Chung1, Sung-Ho Cha1, Deog-Yoon Kim2
1Department of Pediatrics, College of Medicine, Kyung Hee University, Seoul, Korea
2Department of Nuclear Medicine, College of Medicine, Kyung Hee University, Seoul, Korea
남매에서 발생한 Rotor 증후군 2례
박화진1, 김은성1, 정지영1, 차성호1, 김덕윤2
1경희대학교 의과대학 소아과학교실
2경희대학교 의과대학 핵의학교실
Correspondence: 
Sung-Ho Cha,Email: tcha0319@pednet.co.kr
Abstract
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.
Key Words: Rotor syndrome, Hyperbilirubinemia




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