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Dubin-Johnson综合征的临床特点分析

姬蒙 张金平 李建生

引用本文:
Citation:

Dubin-Johnson综合征的临床特点分析

详细信息
  • 中图分类号: R596

Clinical features of Dubin-Johnson syndrome

  • 摘要:

    Dubin-Johnson综合征(DJS)是一种少见的常染色体隐性遗传病,是由于毛细胆管上特异性有机阴离子转运蛋白基因缺陷,导致结合胆红素排泄障碍,血液中结合胆红素升高,黑色色素在肝细胞内沉着,大体表现为黑肝。该疾病预后良好,不需特殊治疗,但容易被误诊导致患者反复就诊治疗,承担不必要的精神和经济负担。近年来有报道本病可合并多种疾病,诊断较困难,从而对持续性黄疸难以解释,并且患者可在中老年时期就诊,而不是在青少年好发时期,容易遗漏该疾病导致误诊。本文对DJS的发病机制、临床特点及近年来的诊断方法等作一综述。

     

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  • 出版日期:  2013-04-20
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