RESUMO
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.
Assuntos
Adulto , Humanos , Masculino , Sistema Biliar , Bilirrubina , Colecistografia , Vesícula Biliar , Doença de Gilbert , Hiperbilirrubinemia , Hiperbilirrubinemia Hereditária , Hiperpigmentação , Icterícia , Icterícia Idiopática Crônica , Fígado , Linfoma , Exame Físico , Esclera , Neoplasias Cutâneas , Taxa de Sobrevida , Disofenina Tecnécio Tc 99mRESUMO
Approximately half of the patients with systemic lupus erythematosus(SLE) develop localized or diffuse lymphadenopathy during the evolution of the disease. But hilar and mediastinal lymph node enlargement due to SLE is rare. The histologic findings of lupus lymphadenitis range from nonspecific lymphadenitis with variable degrees of cellular necrosis to the less common but more specific appearance of extensive necrosis. Advanced lesions are characterized by hematoxylin bodies and condensation of DNA on the vessel walls. Hematoxylin bodies are amorphous homogeneous violet or lilac colored structures composed of partially depolymerized DNA, mixed with protein, carbohydrates and globulins which may be seen deposited on vessel walls and within the paracortex of lymph node. They are virtually diagnostic of SLE and would exclude malignant lymphoma. SLE with mediastinal lymphadenopathy is rare so we report this case with a review of literatures, which was diagnosed by hematoxylin bodies on pathologin findings of cervical lymph node.