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Publication Name World Journal of Hepatology
Manuscript ID 108253
Country United Arab Emirates
Category Gastroenterology & Hepatology
Manuscript Type Minireviews
Article Title New hope in treating progressive familial intrahepatic cholestasis in children
Manuscript Source Invited Manuscript
All Author List Lama Ebrahim Mkarem, Mohammed Ali Hosny Batika and Rana R Bitar
Funding Agency and Grant Number
Corresponding Author Rana R Bitar, Assistant Professor, Division of Pediatric Gastroenterology, Sheikh Khalifa Medical City, Al Karamah St., Abu Dhabi 51900, United Arab Emirates. rahmad@seha.ae
Key Words Progressive familial intrahepatic cholestasis; Odevixibat; Intestinal bile acid transporter inhibitor; Maralixibat
Core Tip Progressive familial intrahepatic cholestasis is a heterogenous autosomal recessive progressive cholestatic liver disease with varying genotype and phenotype. Identifying the underlying genetic disorder allows better understanding of the clinical phenotype and associated complications. Management is aimed at nutritional support, trying to make bile more hydrophilic by administering ursodeoxycholic acid and control of pruritus. Surgical biliary diversion has proved to reduce bile acid levels and improve pruritis in some patients. New hope exists with ileal bile acid transporter receptor inhibitors, however more data is still required. The ultimate treatment for end stage liver disease and persistent pruritus is liver transplantation.
Citation Mkarem LE, Batika MAH, Bitar RR. New hope in treating progressive familial intrahepatic cholestasis in children. World J Hepatol 2025; In press
Received
2025-04-09 02:03
Peer-Review Started
2025-04-09 02:03
To Make the First Decision
Return for Revision
2025-04-25 13:02
Revised
2025-04-25 20:59
Second Decision
2025-06-23 02:49
Accepted by Journal Editor-in-Chief
Accepted by Executive Editor-in-Chief
2025-06-23 07:36
Articles in Press
2025-06-23 07:36
Publication Fee Transferred
Edit the Manuscript by Language Editor
Typeset the Manuscript
ISSN 1948-5182 (online)
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 NonCommercial (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: http://creativecommons.org/licenses/by-nc/4.0/
Copyright ©The Author(s) 2025. Published by Baishideng Publishing Group Inc. All rights reserved.
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