Korean J Pediatr Gastroenterol Nutr.
2002 Mar;5(1):101-107.
Two Cases of Rotor Syndrome in Siblings
- Affiliations
-
- 1Department of Pediatrics, Gil Hospital, Gachon Medical School, Incheon, Korea. onecar@ghil.com
- 2Department of Nuclear Medicine, Gachon Medical School, Incheon, Korea.
- 3Department of Pathology, Gil Hospital, Gachon Medical School, Incheon, Korea.
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.
