Review
Copyright ©The Author(s) 2023. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Hepatol. Oct 27, 2023; 15(10): 1109-1126
Published online Oct 27, 2023. doi: 10.4254/wjh.v15.i10.1109
Challenges and dilemmas in pediatric hepatic Wilson’s disease
Upasana Ghosh, Moinak Sen Sarma, Arghya Samanta
Upasana Ghosh, Department of Pediatric Gastroenterology, Sanjay Gandhi Post graduate Institute of Medical Sciences, Lucknow 226014, Uttar Pradesh, India
Moinak Sen Sarma, Arghya Samanta, Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow 226014, India
Author contributions: Ghosh U contributed to the literature review, analysis and drafting of the initial manuscript; Sen Sarma M contributed to the conception and design of the manuscript and critical revision of the initial manuscript; Samanta A contributed to the interpretation of the data and critical revision of the initial manuscript.
Conflict-of-interest statement: All authors declare no conflict of interest.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Moinak Sen Sarma, MBBS, MD, Adjunct Associate Professor, Doctor, Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Raebareli Road, Lucknow 226014, India. moinaksen@yahoo.com
Received: July 31, 2023
Peer-review started: July 31, 2023
First decision: August 22, 2023
Revised: September 23, 2023
Accepted: October 8, 2023
Article in press: October 8, 2023
Published online: October 27, 2023
Core Tip

Core Tip: Wilson’s disease is an important and common cause of chronic liver disease among children with multi-organ affection. There are various biochemical parameters that aid in making the diagnosis but are nonetheless fraught with limitations. Treatment is lifelong and it’s important to maintain adequate chelation while monitoring for its adverse effects. It is important to distinguish the adverse effects of chelators from the disease manifestations per se, as the management is diagonally opposite, for example, differentiating D-penicillamine-induced paradoxical neurological worsening from neurological manifestations of Wilson disease. This review discusses these challenges in making a diagnosis and treating pediatric hepatic Wilson’s disease.