Liver transplantation in neurological Wilson’s disease in children: Where do we stand?
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.
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BibTeX
@article{ghosh2026liver,
title = {{Liver transplantation in neurological Wilson’s disease in children: Where do we stand?}},
author = {Upasana Ghosh and Nalini Kanta Ghosh},
journal = {World Journal of Clinical Pediatrics},
year = {2026},
month = nov,
doi = {10.5409/wjcp.115258},
url = {https://doi.org/10.5409/wjcp.115258},
note = {CC BY-NC 4.0},
}
RIS
TY - JOUR
TI - Liver transplantation in neurological Wilson’s disease in children: Where do we stand?
AU - Upasana Ghosh
AU - Nalini Kanta Ghosh
PY - 2026
DA - 2026/11/03
JO - World Journal of Clinical Pediatrics
DO - 10.5409/wjcp.115258
UR - https://doi.org/10.5409/wjcp.115258
AB - 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.
SN - 2219-2808
ER -