Diagnosis and management of Alagille and progressive familial intrahepatic cholestasis.

Abstract

Alagille syndrome and progressive familial intrahepatic cholestasis are conditions that can affect multiple organs. Advancements in molecular testing have aided in the diagnosis of both. The impairment of normal bile flow and secretion leads to the various hepatic manifestations of these diseases. Medical management of Alagille syndrome and progressive familial intrahepatic cholestasis remains mostly targeted on supportive care focusing on quality of life, cholestasis, and fat-soluble vitamin deficiency. The most difficult therapeutic issue is typically related to pruritus, which can be managed by various medications such as ursodeoxycholic acid, rifampin, cholestyramine, and antihistamines. Surgical operations were previously used to disrupt enterohepatic recirculation, but recent medical advancements in the use of ileal bile acid transport inhibitors have shown great efficacy for the treatment of pruritus in both Alagille syndrome and progressive familial intrahepatic cholestasis.

• Authors: Katherine Cheng, Philip Rosenthal
• Journal: Hepatology communications (Hepatol Commun) Vol. 7 Issue 12 (Dec 01 2023) ISSN: 2471-254X [Electronic] United States
• PMID: 38055640 (Publication Type: Journal Article)
• Copyright: Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Association for the Study of Liver Diseases.

Topics

• Humans
• Alagille Syndrome (diagnosis, genetics, therapy)
• Quality of Life
• Cholestasis
• Pruritus (diagnosis, surgery)