Korean J Med.
2000 Jul;59(1):109-113.
A case of rotor syndrome
- Affiliations
-
- 1Department of Internal Medicine, Seoul Red Cross Hospital, Seoul, Korea. hahnbc@channeli.net
- 2Department of Internal Medicine, Asan Medical Center, Ulsan University College of Medicine, Seoul, Korea.
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.
Keyword
MeSH Terms
-
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
Bilirubin
Technetium Tc 99m Disofenin
