In recent years, great progress in the research on the hereditary intrahepatic cholestasis have achieved.In this article, we reviewed the pathogenesis especially the hereditary mechanism of ICP.

• 近年来人们对遗传性肝内胆汁淤积性疾病的研究取得了较大的进展,发现了若干与胆汁淤积有关的基因。