A case of rotor syndrome.
- Author:
Jin Hwi KIM
1
;
Yang Suh KOO
;
Jong Ik JEONG
;
Sang Yong JEONG
;
Duk Ho KWUN
;
Dong Woo SHIN
;
Byung Chul HAHN
;
Dong Jin SUH
Author Information
1. Department of Internal Medicine, Seoul Red Cross Hospital, Seoul, Korea. hahnbc@channeli.net
- Publication Type:Case Report
- Keywords:
Skin neoplasm;
Lymphoma;
Survival rate
- MeSH:
Adult;
Biliary Tract;
Bilirubin;
Cholecystography;
Gallbladder;
Gilbert Disease;
Humans;
Hyperbilirubinemia;
Hyperbilirubinemia, Hereditary*;
Hyperpigmentation;
Jaundice;
Jaundice, Chronic Idiopathic;
Liver;
Lymphoma;
Male;
Physical Examination;
Sclera;
Skin Neoplasms;
Survival Rate;
Technetium Tc 99m Disofenin
- From:Korean Journal of Medicine
2000;59(1):109-113
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Rotor syndrome is a rare benign familial disorder characterized by chronic, fluctuating, nonhemolytic and predominantly conjugated hyperbilirubinemia with normal liver tissue. In contrast to Dubin-Johnson syndrome, there is no liver hyperpigmentation in Rotor syndrome, and BSP clearance does not show a secondary retention peak. The serum bilirubin in patients with Gilbert's syndrome is almost all unconjugated in contrast to Rotor syndrome. A 29-year-old male was admitted due to persistent jaundice. Physical examination revealed icteric sclera without hepatosplenomegaly. Laboratory findings showed increased serum bilirubin with indirect bilirubin predominance. Urinary excretion of total coproporphyrin was markedly elevated, and coproporphyrin I was 66% of total urinary coproporphyrin. Oral cholecystography showed well visualized the gallbladder, but 99mTc-DISIDA scan showed markedly decreased hepatic uptake and poor visualization of the gallbladder and biliary tract. Histology of the liver showed no abnormal finding. We report the case with the review of literature.
