RESUMEN
Rotor syndrome is a rare disease of hereditary hyperbilirubinemia transmitted with autosomal recessive trait. In general, Rotor syndrome shows direct hyperbilirubinemia and there has been several reports since Sons's report in 1966, in Korea. A 34-year-old female was admitted with the chief complaint of intermittent icteric sclera for 24 years. There was no family history of jaundice. Rotor syndrome was diagnosed by oral cholecystogram, BSP retention test, 99mTc-DISIDA scan, liver biopsy and electron microscopy study of liver biopsy specimen. We report this case with brief review of the literature.
Asunto(s)
Adulto , Femenino , Humanos , Biopsia , Hiperbilirrubinemia , Hiperbilirrubinemia Hereditaria , Ictericia , Corea (Geográfico) , Hígado , Microscopía Electrónica , Enfermedades Raras , Esclerótica , Disofenina de Tecnecio Tc 99mRESUMEN
Intrapulmonary shunt measured by (99m)Tc-MAA perfusion lung scan. The study was included 76 patients. Significant amount of intrapulmonary shunt was observed in the pulmonary disease patients and liver disease patients. The shunt amount was correlated well with PaO2 and AaDO2 level. Further study is needed to search the various factors affecting the amount of intrapulmonary shunt.