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Copyright ©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Hepatol. Nov 27, 2021; 13(11): 1552-1567
Published online Nov 27, 2021. doi: 10.4254/wjh.v13.i11.1552
Chelation therapy in liver diseases of childhood: Current status and response
Jayendra Seetharaman, Moinak Sen Sarma
Jayendra Seetharaman, Moinak Sen Sarma, Department of Pediatric Gastroenterology, Sanjay Gandhi Post-graduate Institute of Medical Sciences, Lucknow 226014, Uttar Pradesh, India
Author contributions: Seetharaman J primarily drafted the manuscript; Sen Sarma M provided the conception and revised the manuscript.
Conflict-of-interest statement: The authors declare that there are no conflicts 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: http://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Moinak Sen Sarma, MD, DM Associate Professor, Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Raebareli Road, Lucknow 226014, Uttar Pradesh, India. moinaksen@gmail.com
Received: February 27, 2021
Peer-review started: February 27, 2021
First decision: May 2, 2021
Revised: May 7, 2021
Accepted: August 20, 2021
Article in press: August 20, 2021
Published online: November 27, 2021
Processing time: 269 Days and 16 Hours
Abstract

Chelation is the mainstay of therapy in certain pediatric liver diseases. Copper and iron related disorders require chelation. Wilson’s disease (WD), one of the common causes of cirrhosis in children is treated primarily with copper chelating agents like D-penicillamine and trientine. D-Penicillamine though widely used due its high efficacy in hepatic WD is fraught with frequent adverse effects resulting discontinuation. Trientine, an alternative drug has comparable efficacy in hepatic WD but has lower frequency of adverse effects. The role of ammonium tetra-thiomolybdate is presently experimental in hepatic WD. Indian childhood cirrhosis is related to excessive copper ingestion, rarely seen in present era. D-Penicillamine is effective in the early part of this disease with reversal of clinical status. Iron chelators are commonly used in secondary hemochromatosis of liver in hemolytic anemias. There are strict chelation protocols during bone marrow transplant. The role of iron chelation in neonatal hemochromatosis is presently not in vogue due to its poor efficacy and availability of other modalities of therapy. Hereditary hemochromatosis is rare in children and the use of iron chelators in this condition is limited.

Keywords: Wilson’s disease; D-Penicillamine; Trientine; Indian childhood cirrhosis; Deferoxamine; Deferasirox; Hemochromatosis

Core Tip: Chelation forms the most important part of management of certain liver diseases in children. In Wilson's disease and secondary hemochromatosis related to transfusion, chelation is well established treatment modality with proven efficacy. In other diseases like copper associated childhood cirrhosis and neonatal hemochromatosis the role of chelation is doubtful. In hereditary hemochromatosis, chelation is recommended as alternative therapy. The selection of chelating agents for treatment depends on the efficacy, feasibility and risk of adverse effects known from literature. The review discusses the concepts of chelation and reviews the literature to assess the role of chelation in treatment of various pediatric liver diseases.