作者: Aaron J. Small , Konstantinos N. Lazaridis
DOI: 10.1007/978-1-59745-118-5_6
关键词: Pathology 、 Medicine 、 Cholestasis 、 Alagille syndrome 、 Benign Recurrent Intrahepatic Cholestasis 、 Progressive familial intrahepatic cholestasis 、 Aagenaes syndrome
摘要: Cholestasis is an important manifestation of a variety liver diseases. Intrahepatic cholestasis can result from genetic defects epithelial cells. Molecular studies in humans have provided insight into rare cholestatic syndromes such as Alagille syndrome, progressive familial intrahepatic cholestasis, benign recurrent and Aagenaes syndrome to name few. Further characterization these disorders the defective genes associated with them will aid understanding hepatobiliary biology other more common causes cholestasis.