Rare Causes of Cholestasis

作者: Aaron J. Small , Konstantinos N. Lazaridis

DOI: 10.1007/978-1-59745-118-5_6

关键词: PathologyMedicineCholestasisAlagille syndromeBenign Recurrent Intrahepatic CholestasisProgressive familial intrahepatic cholestasisAagenaes 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.

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