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World J Hepatol. Nov 27, 2021; 13(11): 1707-1726
Published online Nov 27, 2021. doi: 10.4254/wjh.v13.i11.1707
Mitochondrial hepatopathy: Respiratory chain disorders- ‘breathing in and out of the liver’
Amrit Gopan, Moinak Sen Sarma
Amrit Gopan, Department of Gastroenterology, Seth G.S Medical College and K.E.M Hospital, Mumbai 400012, India
Moinak Sen Sarma, Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow 226014, India
Author contributions: Gopan A conducted literature review, wrote the initial draft, and designed figures and tables; Sarma MS conceptualized the study, framed the design of the paper, and did critical revisions in structuring the final manuscript; Both authors have read and approved the final document.
Conflict-of-interest statement: Gopan A and Sarma MS declare no conflict of interest related to this publication.
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, DM, Associate Professor, Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Rae Bareilly Road, Lucknow 226014, India. moinaksen@gmail.com
Received: May 24, 2021
Peer-review started: May 24, 2021
First decision: June 15, 2021
Revised: June 30, 2021
Accepted: August 18, 2021
Article in press: August 18, 2021
Published online: November 27, 2021
Processing time: 183 Days and 16.5 Hours
Core Tip

Core Tip: Liver disease with multi-system involvement should arouse the suspicion for mitochondrial respiratory chain hepatopathies. These disorders are predominantly autosomal recessive with some having a maternal inheritance. Presence of lactic acidosis without hypoglycemia is an important clue. A tiered evaluation yields the most data, with the final step being a genetic and enzyme analysis from tissue of interest. Treatment is largely supportive with blood transfusions, correction of acidosis and shock, providing cofactors and salvage therapies, with liver transplantation in a select group. A periodic follow-up is mandatory for monitoring evolution of disease including “migration” to other systems.