BPG is committed to discovery and dissemination of knowledge
Articles Published Processes
9/18/2026 3:45:44 AM | Browse: 1 | Download: 0
 |
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 |
|
2026-08-03 06:49 |
|
Publish the Manuscript Online |
|
2026-09-18 03:45 |
| 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. |
| Article Reprints |
For details, please visit: http://www.wjgnet.com/bpg/gerinfo/247
|
| Permissions |
For details, please visit: http://www.wjgnet.com/bpg/gerinfo/207
|
| Publisher |
Baishideng Publishing Group Inc, 7041 Koll Center Parkway, Suite 160, Pleasanton, CA 94566, USA |
| Website |
http://www.wjgnet.com |
| 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 |
| ORCID |
|
| 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. |
| Publish Date |
2026-09-18 03:45 |
| Citation |
Islek A, İşlek SK, Tumgor G. Progressive familial intrahepatic cholestasis: From childhood to adulthood. World J Gastroenterol 2026; 32(41): 121544
|
| URL |
https://www.wjgnet.com/1007-9327/full/v32/i41/121544.htm |
| DOI |
https://doi.org/10.3748/wjg.121544 |
Copyright © 1993-2026 Baishideng Publishing Group Inc, 7041 Koll Center Parkway, Suite 160, Pleasanton, CA 94566, USA. All rights reserved, including rights relating to text and data mining, AI training, and similar technologies. For open-access content, the applicable copyright and licensing terms govern.
