Two Cases of Rotor Syndrome in Siblings / 소아과
Korean Journal of Pediatrics
;
: 892-895, 2004.
Article
in Korean
| WPRIM
| ID: wpr-148790
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.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Physical Examination
/
Sclera
/
Biliary Tract
/
Bilirubin
/
Technetium Tc 99m Disofenin
/
Siblings
/
Gallbladder
/
Hepatomegaly
/
Hyperbilirubinemia, Hereditary
/
Hyperbilirubinemia
Limits:
Humans
Language:
Korean
Journal:
Korean Journal of Pediatrics
Year:
2004
Type:
Article
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