All issues > Volume 47(8); 2004

Case Report

Korean J Pediatr. 2004;47(8):892-895. Published online August 15, 2004.

Two Cases of Rotor Syndrome in Siblings

Hwa-Jin HJ Park¹, Eun-Sung ES Kim¹, Ji-Young JY Chung¹, Sung-Ho SH Cha¹, Deog-Yoon DY Kim²

¹Department of Pediatrics, College of Medicine, Kyung Hee University, Seoul, Korea
²Department of Nuclear Medicine, College of Medicine, Kyung Hee University, Seoul, Korea

Correspondence Sung-Ho SH 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.

Keywords :Rotor syndrome, Hyperbilirubinemia