형제에서 발생한 Rotor 증후군 2례
Two Cases of Rotor Syndrome in Siblings
김용국, 이정복, 임혜라, 류일, 차한, 이학수, 김종호, 정동해,
소속 상세정보
김용국 ( Kim Yong-Kuk )
가천의과학대학교 소아과학교실
이정복 ( Lee Jung-Bok )
가천의과학대학교 소아과학교실
임혜라 ( Lim Hae-Ra )
가천의과학대학교 소아과학교실
류일 ( Ryoo Eell )
가천의과학대학교 소아과학교실
차한 ( Tchah Hann )
가천의과학대학교 소아과학교실
이학수 ( Lee Hak-Soo )
가천의과학대학교 소아과학교실
김종호 ( Kim Jong-Ho )
가천의과학대학교 핵의학과
정동해 ( Chung Dong-Hae )
가천의과학대학교 병리과
KMID : 0816120020050010101
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.
키워드
Rotor syndrome;Siblings
원문 및 링크아웃 정보
등재저널 정보