Sivakumar M, Powell LW. Management of human factors engineering-associated hemochromatosis: A 2015 update. World J Hepatol 2016; 8(8): 395-400 [PMID: 27004087 DOI: 10.4254/wjh.v8.i8.395]
Corresponding Author of This Article
Lawrie W Powell, MD, PhD, Director, Professor, Centre for the Advancement of Clinical Research, Royal Brisbane and Women’s Hospital Campus, Level 4, UQ Centre for Clinical Research, Building 71/918, Brisbane QLD 4029, Australia. lawrie.powell@qimrberghofer.edu.au
Research Domain of This Article
Gastroenterology & Hepatology
Article-Type of This Article
Minireviews
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/
World J Hepatol. Mar 18, 2016; 8(8): 395-400 Published online Mar 18, 2016. doi: 10.4254/wjh.v8.i8.395
Management of human factors engineering-associated hemochromatosis: A 2015 update
Menaka Sivakumar, Lawrie W Powell
Menaka Sivakumar, School of Medicine, the University of Queensland, Brisbane QLD 4029, Australia
Lawrie W Powell, Centre for the Advancement of Clinical Research, Royal Brisbane and Women’s Hospital Campus, Brisbane QLD 4029, Australia
Author contributions: Both authors contributed equally writing the review.
Conflict-of-interest statement: Neither author has any conflict of interest to declare (including but not limited to commercial, personal, political, intellectual, or religious interests).
Open-Access: 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/
Correspondence to: Lawrie W Powell, MD, PhD, Director, Professor, Centre for the Advancement of Clinical Research, Royal Brisbane and Women’s Hospital Campus, Level 4, UQ Centre for Clinical Research, Building 71/918, Brisbane QLD 4029, Australia. lawrie.powell@qimrberghofer.edu.au
Telephone: +61-7-36462352 Fax: +61-7-36462355
Received: August 7, 2015 Peer-review started: August 10, 2015 First decision: September 21, 2015 Revised: January 27, 2016 Accepted: March 7, 2016 Article in press: March 9, 2016 Published online: March 18, 2016 Processing time: 221 Days and 5.6 Hours
Core Tip
Core tip: The concept of hemochromatosis as a single disease entity has changed to an iron storage disease resulting from several genetic disorders although the final common metabolic pathway is inappropriate iron absorption from the intestine and progressive tissue iron loading. The most common form of the disease is due to a mutation in the human factors engineering gene resulting in cysteine tyrosine substitution at position 282 in the molecule. This mutation is relatively common in populations of northern European extraction but is rare in other populations. In contrast other rarer forms of hemochromatosis resulting from other mutations in the hepcidin pathway are quite ubiquitous. The main stay of treatment remains venesection although new oral iron-chelating agents show promise.
