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Articles in Press
6/11/2026 7:58:47 AM | Browse: 3 | Download: 4
| Category |
Pediatrics |
| Manuscript Type |
Minireviews |
| Article Title |
Wilson disease in children: Recent update on pathophysiology and management
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| Manuscript Source |
Invited Manuscript |
| All Author List |
Khan Lamia Nahid, Mohammad Rukunuzzaman, Rubaiyat Alam and Fahmida Begum |
| Funding Agency and Grant Number |
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| 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
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| PDF |
121660-in-press.pdf
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Received |
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2026-03-30 07:27 |
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Peer-Review Started |
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2026-03-31 07:08 |
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First Decision by Editorial Office Director |
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2026-04-10 08:10 |
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Return for Revision |
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2026-04-10 08:10 |
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Revised |
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2026-05-09 05:06 |
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Publication Fee Transferred |
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Second Decision by Editor |
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2026-06-11 02:39 |
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Second Decision by Editor-in-Chief |
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Final Decision by Editorial Office Director |
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2026-06-11 07:58 |
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Articles in Press |
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2026-06-11 07:58 |
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Edit the Manuscript by Language Editor |
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Typeset the Manuscript |
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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. |
| Permissions |
For details, please visit: http://www.wjgnet.com/bpg/gerinfo/207
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| Publisher |
Baishideng Publishing Group Inc, 7041 Koll Center Parkway, Suite 160, Pleasanton, CA 94566, USA |
| Website |
http://www.wjgnet.com |
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