基础医学与临床 ›› 2010, Vol. 30 ›› Issue (9): 988-990.

• 临床园地 • 上一篇    下一篇

良性复发性肝内胆汁淤积

陈东文 吴东 孙晓红 朱丽明 孙钢   

  1. 中国医学科学院北京协和医学院北京协和医院 中国医学科学院北京协和医学院北京协和医院 中国医学科学院北京协和医学院北京协和医院消化内科
  • 收稿日期:2009-12-30 修回日期:2010-01-25 出版日期:2010-09-05 发布日期:2010-09-05
  • 通讯作者: 吴东

Benign recurrent intrahepatic cholestasis

Dong-wen CHEN, Dong WU, Xiao-hong SUN, Li-ming ZHU, Gang SUN   

  1. PUMC Hospital, CAMS&PUMC PUMC Hospital, CAMS&PUMC
  • Received:2009-12-30 Revised:2010-01-25 Online:2010-09-05 Published:2010-09-05
  • Contact: Dong WU,

摘要: 目的 对国内外良性复发性肝内胆汁淤积(BRIC)病例进行系统性的回顾、归纳及总结。方法 报道北京协和医院的1例BRIC,在国内外文献中检索符合条件的BRIC病例,并对该病的临床特点进行总结。结果 国内有6例符合检索条件,国外21例。BRIC表现为反复发作性的黄疸和皮肤瘙痒,在发作期呈肝内胆汁淤积性黄疸表现,发作间期临床表现,生化指标,影像学表现完全正常。本病预后良好,极少数病例转变为预后不佳的进展性家族性肝内淤积性黄疸(PFIC)。

关键词: 良性复发性肝内淤积, 黄疸, 瘙痒

Abstract: Objective We try to make a thorough and systemic review on benign recurrent intrahepatic cholestais (BRIC). Methods We made a brief introduction on 1 case of BRIC in Peking Union Medical College Hospital and then browsed qualified BRIC cases on internet databases. Result We found 6 cases in China and 21 cases from abroad. BRIC is characteristic by recurrent cholestasis and pruritus with normal manifestation, biochemical sign and imaging during the interval. The clinical course in most cases is benign, with relatively few cases develop progressive familial intrahepatic cholestasis (PFIC) and have a bad prognosis.

Key words: benign recurrent intrahepatic cholestasis, jaundice, pruritus