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Publication Name World Journal of Clinical Pediatrics
Manuscript ID 119970
Country United Arab Emirates
Category Cardiac & Cardiovascular Systems
Manuscript Type Evidence Review
Article Title Fontan-related protein-losing enteropathy: A comprehensive review of pathophysiology and current therapies
Manuscript Source Invited Manuscript
All Author List Eman Al Atrash, Doaa Zourob, Huda Alghfeli and Antoine AbdelMassih
Funding Agency and Grant Number
Corresponding Author Eman Al Atrash, MD, Division of Pediatric Gastroenterology, Department of Pediatrics, Mediclinic Airport Hospital, Airport Road, Abu Dhabi 971, United Arab Emirates. emanalatrash8@gmail.com
Key Words Protein-losing enteropathy; Hypoalbuminemia; Intestinal lymphangiectasia; Fontan circulation; Lymphatic disorders
Core Tip Fontan-associated protein-losing enteropathy is a rare but life-threatening complication of Fontan circulation, driven by abnormal hemodynamics, lymphatic dysfunction, and intestinal barrier injury. This review synthesizes current understanding of protein-losing enteropathy pathophysiology and highlights contemporary management strategies, including nutritional optimization, targeted pharmacologic therapies, and emerging lymphatic interventions. Particular emphasis is placed on individualized, mechanism-based treatment and the evolving role of advanced imaging and interventional techniques. Improved recognition and multidisciplinary care have enhanced outcomes; however, refractory disease remains challenging, underscoring the need for novel therapeutic approaches.
Citation Al Atrash E, Zourob D, Alghfeli H, AbdelMassih A. Fontan-related protein-losing enteropathy: A comprehensive review of pathophysiology and current therapies. World J Clin Pediatr 2026; In press
Received
2026-02-12 01:22
Peer-Review Started
2026-02-12 01:27
First Decision by Editorial Office Director
2026-02-25 08:01
Return for Revision
2026-02-25 08:01
Revised
2026-02-26 21:13
Publication Fee Transferred
Second Decision by Editor
2026-05-07 03:24
Second Decision by Editor-in-Chief
Final Decision by Editorial Office Director
2026-05-07 09:59
Articles in Press
2026-05-07 09:59
Edit the Manuscript by Language Editor
Typeset the Manuscript
ISSN 2219-2808 (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.
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