Journal List > Korean J Pediatr Gastroenterol Nutr > v.5(1) > 1110447

Korean J Pediatr Gastroenterol Nutr. 2002 Mar;5(1):101-107. Korean.
Published online Mar 31, 2002.
Copyright © 2002 The Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition
Two Cases of Rotor Syndrome in Siblings
Yong Kuk Kim, Jung Bok Lee, Hae Ra Im, Eell Ryoo, Hann Tchah, Hak Soo Lee, Jong Ho Kim,1 and Dong Hae Jung2
Department of Pediatrics, Gil Hospital, Gachon Medical School, Incheon, Korea.
1Department of Nuclear Medicine, Gachon Medical School, Incheon, Korea.
2Department of Pathology, Gil Hospital, Gachon Medical School, Incheon, Korea.

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.

Keywords: Rotor syndrome; Siblings