Korean J Gastroenterol.
2007 Apr;49(4):251-255.
A Case with Rotor Syndrome in Hyperbilirubinemic Family
- Affiliations
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- 1Department of Internal Medicine, Kyungpook National University School of Medicine, Daegu, Korea. wytak@mail.knu.ac.kr
Abstract
- Rotor syndrome is a rare, benign familial disorder characterized by chronic fluctuating, nonhemolytic and predominantly conjugated hyperbilirubinemia with normal hepatic histology. In contrast to Dubin-Johnson syndrome, there is no liver pigmentation in Rotor syndrome. A 36-year-old man was admitted due to asymptomatic persistent jaundice. His siblings had jaundice with direct hyperbilirubinemia. Physical examination revealed icteric sclerae without hepatosplenomegaly. Laboratory findings showed increased serum bilirubin with direct bilirubinmia. Hepatic uptake and storage capacity of indocyanine green was markedly reduced, while excretion into bile was slightly suppressed. Markedly decreased hepatic uptake and poor visualization of the gallbladder and biliary tract were shown in 99mTc-DISIDA scan. Histology of the liver showed mild steatosis without pigmentation. We report a case with the review of literature.
Keyword
MeSH Terms
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Adult
Coloring Agents/*diagnostic use/pharmacokinetics
Humans
Hyperbilirubinemia, Hereditary/diagnosis/genetics/radionuclide imaging
Indocyanine Green/*diagnostic use/pharmacokinetics
Jaundice, Chronic Idiopathic/*diagnosis/radionuclide imaging
Liver/radionuclide imaging
Liver Function Tests
Male
Radiopharmaceuticals/*diagnostic use
Technetium Tc 99m Disofenin/*diagnostic use
