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©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Hepatol. Jan 27, 2022; 14(1): 180-194
Published online Jan 27, 2022. doi: 10.4254/wjh.v14.i1.180
Published online Jan 27, 2022. doi: 10.4254/wjh.v14.i1.180
Mitochondrial hepatopathy: Anticipated difficulties in management of fatty acid oxidation defects and urea cycle defects
Aathira Ravindranath, Division of Pediatric Gastroenterology, Institute of Gastrointestinal Sciences, Apollo BGS Hospitals, Mysore 570023, Karnataka, India
Moinak Sen Sarma, Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow 226014, Uttar Pradesh, India
Author contributions: Ravindranath A contributed to the data collection, metadata search, primary drafting of the manuscript; Sarma MS performed the supervision and critical review of the manuscript.
Conflict-of-interest statement: Both the authors have 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: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Moinak Sen Sarma, MD, DM, Associate Professor, Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Rae Bareli Road, Lucknow 226014, Uttar Pradesh, India. moinaksen@yahoo.com
Received: May 21, 2021
Peer-review started: May 21, 2021
First decision: July 27, 2021
Revised: August 19, 2021
Accepted: December 2, 2022
Article in press: December 2, 2021
Published online: January 27, 2022
Peer-review started: May 21, 2021
First decision: July 27, 2021
Revised: August 19, 2021
Accepted: December 2, 2022
Article in press: December 2, 2021
Published online: January 27, 2022
Core Tip
Core Tip: Management of fatty acid oxidation defects and urea cycles defects has to be tailored to specific types of defects. Although dietary intervention remains the most important pillar of successful outcome, role of definitive and potential medications is assuming renewed significance. Management is challenging due to variations in type and severity of the enzyme defect.