Dubin-Johnson综合征(DJS)是肝细胞分泌入胆汁的双亲阴离子轭合物遗传缺陷为其特征的一种常染色体隐性遗传性疾病〔1〕。DJS在1954年由Dubin和Johnson等先后报道,属于一种先天性胆红素代谢障碍性肝病,因临床少见,且易与病毒性肝炎相混淆而被长期误诊。现将1例DJS报告如下。 Dubin-Johnson syndrome (DJS) is an autosomal recessive inherited disorder characterized by genetic defects in the parental anion conjugate secreted into the bile by hepatocytes [1]. DJS was first reported by Dubin and Johnson in 1954. It belongs to a congenital bilirubin metabolic disorder liver disease, which has been misdiagnosed for a long time because of its rare clinical features and easy confusion with viral hepatitis. Now a case of DJS report as follows.