title: [Rotor syndrome. A long-term study].
abstract: A patient with Rotor syndrome has been followed up for 28 years. The subject was in good health and exhibited marked jaundice due to a hyperbilirubinemia ranging between 5 and 8 mg bilirubin per 100 ml, about 70% of the bilirubin giving direct van den Berg reaction. The liver was slightly enlarged and of normal color. Histologically it showed traces of a brown pigment and marked siderosis in the hepatocytes. In addition, mild hypersideremia and hypercholesteremia were observed together with increased amounts of bilirubin and urobilinogen in urine. Menthol glucuronide formation was normal. Hepatic excretion of bilirubin, sulfobromophthalein and radio-opaque dyes was impaired. There was no change in liver histology of biopsies taken at an interval of 6 years, the siderosis in particular remaining constant. During the observation a moderate increase in the activities of serum transaminases occurred but no longer recurred when physical and dietetic stress was avoided.
mesh_terms: D000328:Adult; D001652:Bile Ducts; D001663:Bilirubin / Q000097:blood / Q000652:urine; D004322:Drainage; D006801:Humans; D007565:Jaundice / Q000235:genetics; D008099:Liver / Q000648:ultrastructure; D008297:Male; D000637:Transaminases / Q000378:metabolism; D014558:Urobilinogen / Q000652:urine
chemical_list: D014558:Urobilinogen; D000637:Transaminases; D001663:Bilirubin
vernacular_title: Rotor-Syndrom Eine Langzeitbeobachtung