Korean J Pediatr Gastroenterol Nutr.
2004 Sep;7(2):243-247.
A Case of Rotor's Syndrome
- Affiliations
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- 1Department of Pediatrics, Soonchunhyang University Bucheon Hospital, Bucheon, Korea. jop50@schbc.ac.kr
- 2Department of Pathology, Soonchunhyang University Bucheon Hospital, Bucheon, Korea.
Abstract
- Rotor's syndrome is a hereditary disorder characterized by predominantly conjugated hyperbilirubinemia with normal hepatic histology. It resembles Dubin-Johnson syndrome but the main differences are no dark brown pigmentation in the hepatic cells and visualization of the gallbladder in oral cholangiography. We experienced a 14 year-old male patient who had icteric sclerae and predominantly conjugated hyperbilirubinemia when he was hospitalized for varicocelectomy. His liver biopsy specimen showed no dark brown pigmentation and any other pathologic abnormalities in the hepatic cells. Hepatobiliary scan shows no evidence of obstructive lesions. His urinary excretion of total coproporphyrin was markedly increased.
