释义 |
Rotor syndrome
Ro·tor syn·drome (rō-tōr'), jaundice appearing in childhood due to impaired biliary excretion; most of the plasma bilirubin is conjugated, liver function test results are usually normal, and there is no hepatic pigmentation.Rotor syndrome An autosomal recessive (OMIM:237450) form of primary conjugated hyperbilirubinaemia characterised by mild jaundice without haemolysis that begins shortly after birth or in childhood. Affected individuals have delayed plasma clearance of the unconjugated anionic dye bromsulphthalein and high urinary excretion of coproporphyrin I. Hepatic pigmentation is normal. Molecular pathology Defects in SLCO1B1 and SLCO1B3, which encode organic anion transporters, cause Rotor syndrome.Ro·tor syn·drome (rō-tōr' sin'drōm) Jaundice appearing in childhood due to impaired biliary excretion; most of the plasma bilirubin is conjugated, liver function results are usually normal, and there is no hepatic pigmentation. Rotor syndrome Mild JAUNDICE with an apparently normal liver which appears to be temporarily affected in its ability to take up and store BILIRUBIN. The outlook is excellent. (Arturo B. Rotor, Phillipine physician.).Rotor, Arturo B., 20th century Philippine internist. Rotor syndrome - jaundice appearing in childhood due to impaired biliary excretion. |