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Publication Name World Journal of Transplantation
Manuscript ID 111122
Country India
Category Anesthesiology
Manuscript Type Minireviews
Article Title Perioperative management of pediatric patients with inborn errors of metabolism during liver transplantation
Manuscript Source Invited Manuscript
All Author List Susan Paulin, Akila Rajakumar, Jagadeesh Menon, Naresh Shanmugam and Mohamed Rela
Funding Agency and Grant Number
Corresponding Author Akila Rajakumar, FRCA, Head, Department of Liver Transplant Anaesthesia and Intensive Care, Institute of Liver Disease and Transplantation, Rela Institute, 7, CLC Works Road, Chromepet, Chennai 600044, Tamil Nadu, India. drakila.rajakumar@gmail.com
Key Words Inborn errors of metabolism; Anaesthesia for paediatric liver transplantation; Metabolic crisis; Hyperammonemia in paediatric liver transplantation; Perioperative care in metabolic liver diseases
Core Tip Most metabolic disorders are liver-based, and liver transplantation (LT) has emerged as an effective cure for a variety of inherited metabolic disorders in the pediatric population. LT has been shown to offer a cure, deter disease progression and provide symptomatic improvement in patients with inherited metabolic disorders. Perioperative management of these children presenting for LT with inborn errors of metabolism (IEM) requires disease specific and patient specific strategies in all aspects right from preoperative assessment, intraoperative and postoperative management compared to those presenting with other indications. Knowledge about the distribution of deficient enzymes, the percentage of enzymes replaced by LT, and the extent of extrahepatic involvement helps anticipate and manage complications in the perioperative period.
Citation Paulin S, Rajakumar A, Menon J, Shanmugam N, Rela M. Perioperative management of pediatric patients with inborn errors of metabolism during liver transplantation. World J Transplant 2025; In press
Received
2025-06-27 01:09
Peer-Review Started
2025-06-27 01:09
To Make the First Decision
Return for Revision
2025-07-17 10:22
Revised
2025-07-25 08:18
Second Decision
2025-11-05 02:42
Accepted by Journal Editor-in-Chief
Accepted by Executive Editor-in-Chief
2025-11-05 08:51
Articles in Press
2025-11-05 08:51
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Edit the Manuscript by Language Editor
Typeset the Manuscript
ISSN 2220-3230 (online)
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/
Copyright The Author(s) 2025. Published by Baishideng Publishing Group Inc. All rights reserved.
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