Two Cases of Rotor Syndrome in Siblings.
- Author:
Hwa Jin PARK
1
;
Eun Sung KIM
;
Ji Young CHUNG
;
Sung Ho CHA
;
Deog Yoon KIM
Author Information
1. Department of Pediatrics, College of Medicine, Kyung Hee University, Seoul, Korea. tcha0319@pednet.co.kr
- Publication Type:Case Report
- Keywords:
Rotor syndrome;
Hyperbilirubinemia
- MeSH:
Biliary Tract;
Bilirubin;
Gallbladder;
Hepatomegaly;
Humans;
Hyperbilirubinemia;
Hyperbilirubinemia, Hereditary*;
Liver;
Physical Examination;
Sclera;
Siblings*;
Technetium Tc 99m Disofenin
- From:Korean Journal of Pediatrics
2004;47(8):892-895
- CountryRepublic of Korea
- Language:Korean
-
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.
