Two Cases of Rotor Syndrome in Siblings.
- Author:
Yong Kuk KIM
1
;
Jung Bok LEE
;
Hae Ra IM
;
Eell RYOO
;
Hann TCHAH
;
Hak Soo LEE
;
Jong Ho KIM
;
Dong Hae JUNG
Author Information
1. Department of Pediatrics, Gil Hospital, Gachon Medical School, Incheon, Korea. onecar@ghil.com
- Publication Type:Case Report
- Keywords:
Rotor syndrome;
Siblings
- MeSH:
Adolescent;
Bile Ducts;
Bilirubin;
Biopsy;
Child;
Humans;
Hyperbilirubinemia, Hereditary*;
Indocyanine Green;
Injections, Intravenous;
Liver;
Male;
Needles;
Pigmentation;
Plasma;
Sclera;
Siblings*;
Technetium Tc 99m Disofenin;
Transaminases;
Urinary Bladder
- From:Korean Journal of Pediatric Gastroenterology and Nutrition
2002;5(1):101-107
- CountryRepublic of Korea
- Language:Korean
-
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.
