Menon J, Vij M, Sachan D, Rammohan A, Shanmugam N, Kaliamoorthy I, Rela M. Pediatric metabolic liver diseases: Evolving role of liver transplantation. World J Transplant 2021; 11(6): 161-179 [PMID: 34164292 DOI: 10.5500/wjt.v11.i6.161]
Corresponding Author of This Article
Mukul Vij, MD, Doctor, Department of Pathology, Dr Rela Institute and Medical Center, Chromepet, Chennai 600044, Tamil Nadu, India. mukul.vij.path@gmail.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/
Jagadeesh Menon, Ashwin Rammohan, Naresh Shanmugam, Ilankumaran Kaliamoorthy, Mohamed Rela, Institute of Liver Disease and Transplantation, Dr Rela Institute and Medical Center, Chennai 600044, Tamil Nadu, India
Mukul Vij, Department of Pathology, Dr Rela Institute and Medical Center, Chennai 600044, Tamil Nadu, India
Deepti Sachan, Department of Transfusion Medicine, Dr Rela Institute and Medical Center, Chennai 600044, Tamil Nadu, India
Author contributions: Menon J, Vij M, and Sachan D conceptualized the review, performed literature search, and drafting of the manuscript; Vij M, Rammohan A, Shanmugam N, Kaliamoorthy I and Rela M edited and revised the review; all authors read and approved the manuscript for publication.
Conflict-of-interest statement: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
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: Mukul Vij, MD, Doctor, Department of Pathology, Dr Rela Institute and Medical Center, Chromepet, Chennai 600044, Tamil Nadu, India. mukul.vij.path@gmail.com
Received: February 28, 2021 Peer-review started: February 28, 2021 First decision: May 5, 2021 Revised: May 13, 2021 Accepted: June 3, 2021 Article in press: June 3, 2021 Published online: June 18, 2021
Core Tip
Core Tip: Metabolic disorders are important cause of morbidity and mortality in children. Their clinical presentations are varied and include end-stage liver disease, hepatocellular cancer, renal tubular acidosis, seizures, encephalopathy, myopathy etc. Liver transplantation (LT) is a curative option in many metabolic disorders. LT is contraindicated in mitochondrial disorders with significant extrahepatic involvement. A combined liver kidney transplant is needed in disorders where the underlying defects significantly damages both the organs. The outcome of LT is excellent in metabolic disorders. Auxiliary partial orthotopic LT is an attractive option as it provides the defective enzymes keeping the native liver intact and may hold an option of withdrawing immunosuppression in case gene therapy can be offered in future.
