Pediatric Gastroenterology, Hepatology & Nutrition
- Volume 5 Issue 1
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- Pages.101-107
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- 2002
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- 2234-8646(pISSN)
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- 2234-8840(eISSN)
Two Cases of Rotor Syndrome in Siblings
형제에서 발생한 Rotor 증후군 2례
- Kim, Yong-Kuk (Department of Pediatrics, Gil Hospital, Gachon Medical School) ;
- Lee, Jung-Bok (Department of Pediatrics, Gil Hospital, Gachon Medical School) ;
- Im, Hae-Ra (Department of Pediatrics, Gil Hospital, Gachon Medical School) ;
- Ryoo, Eell (Department of Pediatrics, Gil Hospital, Gachon Medical School) ;
- Tchah, Hann (Department of Pediatrics, Gil Hospital, Gachon Medical School) ;
- Lee, Hak-Soo (Department of Pediatrics, Gil Hospital, Gachon Medical School) ;
- Kim, Jong-Ho (Department of Nuclear Medicine, Gil Hospital, Gachon Medical School) ;
- Jung, Dong-Hae (Department of Pathology, Gil Hospital, Gachon Medical School)
- 김용국 (가천의과대학교 소아과) ;
- 이정복 (가천의과대학교 소아과) ;
- 임혜라 (가천의과대학교 소아과) ;
- 류일 (가천의과대학교 소아과) ;
- 차한 (가천의과대학교 소아과) ;
- 이학수 (가천의과대학교 소아과) ;
- 김종호 (가천의과대학교 핵의학과) ;
- 정동해 (가천의과대학교 병리과)
- Received : 2002.03.07
- Accepted : 2002.03.20
- Published : 2002.03.30
Abstract
We experienced two cases of Rotor syndrome in brothers who were a 13 year-old boy and an 11 year-old boy, respectively. They presented with icteric scleras for a few months. Their common laboratory characteristics were as follows: Direct bilirubin was more increased than indirect bilirubin, but aminotransferases were normal. Plasma indocyanine green (ICG) test revealed hepatic excretory defect: plasma ICG concentrations 15 minutes after intravenous injection were 80.45% and 78.28%, respectively. 99mTc-DISIDA Hepatobiliary scan showed that severely decreased hepatic extraction with mild cardiac blood pool, markedly delayed biliary excretion in both intra- & extra- hepatic bile ducts, delayed visualization of gall bladder, and markedly delayed intestinal biliary passage. Needle liver biopsy showed normal hepatic histology without pigmentation.