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Copyright: ©Author(s) 2026. 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.
World J Clin Pediatr. Dec 9, 2026; 15(4): 115258
Published online Dec 9, 2026. doi: 10.5409/wjcp.115258
Liver transplantation in neurological Wilson’s disease in children: Where do we stand?
Upasana Ghosh, Nalini Kanta Ghosh
Upasana Ghosh, Department of Pediatric Gastroenterology, Post Graduate Institute of Child Health, Noida 201303, Uttar Pradesh, India
Nalini Kanta Ghosh, Department of Liver Transplantation and HPB Surgeries, Fortis Hospital, Noida 201301, Uttar Pradesh, India
Author contributions: Ghosh U wrote the initial manuscript draft; Ghosh NK designed the concept of the manuscript; Ghosh U and Ghosh NK designed the outline of the manuscript, did the literature review, and critical revision of the manuscript. All authors have read and approved the final manuscript.
Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article.
Corresponding author: Upasana Ghosh, Assistant Professor, Department of Pediatric Gastroenterology, Post Graduate Institute of Child Health, Sector 30, Noida 201303, Uttar Pradesh, India. ghoshupasana16@gmail.com
Received: October 15, 2025
Revised: December 17, 2025
Accepted: March 16, 2026
Published online: December 9, 2026
Processing time: 342 Days and 5.8 Hours
Abstract

Wilson’s disease (WD) is a genetic disorder caused by a mutation in the gene ATP7B. It results in the pathological deposition of copper mainly in the liver and the brain leading to hepatic and neurological manifestations. Medical management is effective in most patients (approximately 85%). It includes chelators (D-penicillamine and trientine), which are the mainstay of treatment, and zinc salts, which are used to inhibit the gastrointestinal absorption of copper. However, in cases of WD with neurological manifestations, patients take longer time to improve and sometimes have persistent clinical symptoms, and a few develop irreversible neurological damage despite adequate chelation. Generally, liver transplantation (LT) is indicated in acute fulminant hepatic failure or decompensated liver cirrhosis (despite adequate chelation). However, LT is still debatable for severe neurologic WD because of advanced or irreversible brain damage. There is a dearth of literature on the role of LT in neurological WD. This study aims to review the current literature concerning indications, contraindications, and outcomes of liver transplantation among children with neurological WD.

Keywords: ATP7B protein; Liver transplantation; Wilson’s disease; Hepato-neurologic; Children

Core Tip: Liver transplantation (LT) is indicated in Wilson’s disease (WD) in acute fulminant hepatic failure or decompensated liver cirrhosis. However, LT is still debatable for neurologic WD because of advanced or irreversible brain damage. This review aims to study the current literature concerning indications, contraindications, timing and outcomes of LT among children with neurological WD.

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