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、生化指标及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)。
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.Results We found 6 cases in China and 21 cases from abroad.BRIC is characterised 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.
出处
《基础医学与临床》
CSCD
北大核心
2010年第9期988-990,共3页
Basic and Clinical Medicine
关键词
良性复发性肝内胆汁淤积
黄疸
瘙痒
benign recurrent intrahepatic cholestais
jaundice
pruritus