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Publication Name World Journal of Clinical Cases
Manuscript ID 121660
DOI 10.12998/wjcc.121660
Country Bangladesh
Category Pediatrics
Manuscript Type Minireviews
Article Title Wilson disease in children: Recent update on pathophysiology and management
Manuscript Source Invited Manuscript
All Author List Khan Lamia Nahid, Mohammad Rukunuzzaman, Rubaiyat Alam and Fahmida Begum
Funding Agency and Grant Number
Corresponding Author Khan Lamia Nahid, Associate Professor, Paediatric Gastroenterology and Nutrition, Bangladesh Medical University (BMU), Shahbag, Dhaka 1000, Dhaka, Bangladesh. lamianahid@yahoo.com
Key Words Wilson disease; pathophysiology; ATP7B; Copper; Penicillamine
Core Tip Wilson disease (WD) is an autosomal recessive disease caused by mutations in ATP7B gene which has fundamental role in copper metabolism, leading to accumulation of copper in liver and other vital organs. Major functions of ATP7B are incorporation of copper into apoceruloplasmin and the excretion of copper into bile. From simple asymptomatic elevation of liver enzyme to acute liver failure, chronic hepatitis, portal hypertension may be the initial hepatic presentation of this disease. No single test is diagnostic for WD. Initial testing includes ocular slit-lamp examination, 24-hour urinary copper excretion and serum ceruloplasmin. Leipzig scoring system for diagnosis is used widely. Relative exchangeable copper is the new non-invasive biomarker for WD diagnosis with highest sensitivity and specificity. Patients need lifelong chelation therapy until liver transplantation.
Citation Nahid KL, Rukunuzzaman M, Alam R, Begum F. Wilson disease in children: Recent update on pathophysiology and management. World J Clin Cases 2026; In press
PDF 121660-in-press.pdf
Received
2026-03-30 07:27
Peer-Review Started
2026-03-31 07:08
First Decision by Editorial Office Director
2026-04-10 08:10
Return for Revision
2026-04-10 08:10
Revised
2026-05-09 05:06
Publication Fee Transferred
Second Decision by Editor
2026-06-11 02:39
Second Decision by Editor-in-Chief
Final Decision by Editorial Office Director
2026-06-11 07:58
Articles in Press
2026-06-11 07:58
Edit the Manuscript by Language Editor
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ISSN 2307-8960 (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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