Ghosh U, Sen Sarma M, Samanta A. Challenges and dilemmas in pediatric hepatic Wilson’s disease. World J Hepatol 2023; 15(10): 1109-1126 [PMID: PMC10642431 DOI: 10.4254/wjh.v15.i10.1109]
Corresponding Author of This Article
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
Research Domain of This Article
Gastroenterology & Hepatology
Article-Type of This Article
Review
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (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: http://creativecommons.org/licenses/by-nc/4.0/
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 Processing time: 84 Days and 14.6 Hours
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.
