Publication Name	World Journal of Gastroenterology
Manuscript ID	33422
Country	Serbia

Category	Gastroenterology & Hepatology
Manuscript Type	Case Report
Article Title	Metabolically based liver damage pathophysiology in patients with urea cycle disorders - A new hypothesis
Manuscript Source	Unsolicited Manuscript
All Author List	Ivan Ivanovski, Milos Jesic, Ana Ivanovski, Livia Garavelli and Petar Ivanovski
Funding Agency and Grant Number
Corresponding Author	Petar Ivanovski, Department of Pediatrics, University Children’s Hospital, School of Medicine University of Belgrade, Belgrade 11000, Serbia. petar.ivanovski@udk.bg.ac.rs
Key Words	Urea cycle disorder; Citrullinemia; Neonatal liver iron overload; Fumaric acid; Succinic acid; Krebs' cycle; Transferrin; Zinc fumarate supplementation
Core Tip	Underlying pathophysiology of liver dysfunction in urea cycle disorders (UCDs) is still largely elusive. We hypothesize that the key is deficient fumaric acid production in urea cycle in UCDs, which causes several sequentially intertwined metabolic disturbances with the final result of liver iron overload. Providing the hypothesis is correct, neonatal liver damage in patients having UCD can be prevented by the supplementation of pregnant women with fumaric or succinic acid, prepared in the form of iron supplementation pills. After birth, liver damage in patients having UCDs can be prevented by supplementation of these patients with zinc fumarate or zinc succinylate.
Citation	Ivanovski I, Ješić M, Ivanovski A, Garavelli L, Ivanovski P. Metabolically based liver damage pathophysiology in patients with urea cycle disorders - A new hypothesis. World J Gastroenterol 2017; 23(44): 7930-7938

Received	2017-02-08 09:58
Peer-Review Started	2017-02-09 08:36
To Make the First Decision	2017-04-20 13:52
Return for Revision	2017-04-20 16:20
Revised	2017-05-15 16:52
Second Decision	2017-06-16 13:04
Accepted by Journal Editor-in-Chief
Accepted by Company Editor-in-Chief	2017-06-19 09:37
Articles in Press	2017-06-19 09:37
Publication Fee Transferred	2017-09-21 11:14
Edit the Manuscript by Language Editor
Typeset the Manuscript	2017-11-17 02:33

ISSN	1007-9327 (print) and 2219-2840 (online)
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/
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