Publication Name	World Journal of Gastroenterology
Manuscript ID	121544
DOI	10.3748/wjg.121544
Country	Türkiye

Category	Gastroenterology & Hepatology
Manuscript Type	Review
Article Title	Progressive familial intrahepatic cholestasis: From childhood to adulthood
Manuscript Source	Invited Manuscript
All Author List	Ali Islek, Serap Ketenci İşlek and Gokhan Tumgor
Funding Agency and Grant Number
Corresponding Author	Gokhan Tumgor, MD, Professor, Department of Pediatric Gastroenterology, Cukurova University School of Medicine, South Campus Street, Balcalı village, 15/10 Saricam, Adana 01330, Türkiye. gtumgor74@yahoo.com
Key Words	Bile acid transport; Cholestasis; Genotype-phenotype correlation; Ileal bile acid transporter inhibitors; Genetic diagnosis
Core Tip	Progressive familial intrahepatic cholestasis (PFIC) is a genetically and mechanistically diverse group of cholestatic liver disorders whose clinical spectrum spans from childhood well into adulthood. The disease classification has evolved from a numerical system to a mechanism-based framework encompassing defects in canalicular transport, membrane stability, tight junction integrity, intracellular trafficking, and bile acid regulation. This comprehensive review underscores the significance of integrating early genetic testing with biochemical patterns, particularly gamma-glutamyl transferase levels, for accurate diagnosis. Genotype-phenotype correlations and extrahepatic manifestations are essential for prognostic assessment and individualized management. Emerging targeted therapies, including ileal bile acid transporter inhibitors, support a mechanism-driven approach to PFIC.
Citation	Islek A, İşlek SK, Tumgor G. Progressive familial intrahepatic cholestasis: From childhood to adulthood. World J Gastroenterol 2026; In press DOI: https://doi.org/10.3748/wjg.121544
PDF	121544-in-press.pdf

Received	2026-03-27 03:34
Peer-Review Started	2026-03-27 03:36
First Decision by Editorial Office Director	2026-04-17 08:13
Return for Revision	2026-04-17 08:13
Revised	2026-05-25 11:51
Publication Fee Transferred
Second Decision by Editor	2026-06-08 02:38
Second Decision by Editor-in-Chief
Final Decision by Editorial Office Director	2026-06-08 08:03
Articles in Press	2026-06-08 08:03
Edit the Manuscript by Language Editor
Typeset the Manuscript

ISSN	1007-9327 (print) and 2219-2840 (online)
Open Access	This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution-NonCommercial (CC BY-NC 4.0) license. No commercial re-use. See Permissions. Published by Baishideng Publishing Group Inc.
Copyright	©Author(s) (or their employer(s)) 2026. No commercial re-use. See Permissions. Published by Baishideng Publishing Group Inc.
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