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©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Hepatol. Jan 27, 2022; 14(1): 98-118
Published online Jan 27, 2022. doi: 10.4254/wjh.v14.i1.98
Published online Jan 27, 2022. doi: 10.4254/wjh.v14.i1.98
Recent updates on progressive familial intrahepatic cholestasis types 1, 2 and 3: Outcome and therapeutic strategies
Seema Alam, Bikrant Bihari Lal, Department of Pediatric Hepatology, Institute of Liver and Biliary Sciences, New Delhi 110070, India
Author contributions: Alam S contributed to the conceptualization and intellectual input of final draft of manuscript; Lal BB contributed to the primary draft of manuscript, data collection and literature retrieval; and both authors contributed equally to the manuscript.
Conflict-of-interest statement: Seema Alam and Bikrant Bihari Lal declare no conflict of interest.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Seema Alam, MD, Professor, Department of Pediatric Hepatology, Institute of Liver and Biliary Sciences, D-1 Vasant Kunj, New Delhi 110070, India.
Received: May 22, 2021
Peer-review started: May 22, 2021
First decision: July 6, 2021
Revised: July 17, 2021
Accepted: November 30, 2022
Article in press: November 30, 2021
Published online: January 27, 2022
Processing time: 243 Days and 21.5 Hours
Peer-review started: May 22, 2021
First decision: July 6, 2021
Revised: July 17, 2021
Accepted: November 30, 2022
Article in press: November 30, 2021
Published online: January 27, 2022
Processing time: 243 Days and 21.5 Hours
Core Tip
Core Tip: The spectrum of clinical manifestations in progressive familial intrahepatic cholestasis varies from mild to severe leading to end stage liver disease necessitating liver transplantation. Medical therapy forms the mainstay of treatment of pruritus with surgical biliary diversion reserved for refractory cases. Apical sodium bile salt co-transporter inhibitors are among the most promising newer drugs. This article describes the recent advances in understanding the clinical course and emerging therapies.