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Articles in Press
9/21/2022 9:43:01 PM | Browse: 45 | Download: 58
Category |
Gastroenterology & Hepatology |
Manuscript Type |
Minireviews |
Article Title |
Natural history and management of liver dysfunction in lysosomal storage disorders
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Manuscript Source |
Invited Manuscript |
All Author List |
Moinak Sen Sarma and Parijat Ram Tripathi |
Funding Agency and Grant Number |
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Corresponding Author |
Moinak Sen Sarma, MD, Associate Professor, Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Raebareli Road, Lucknow 226014, India. moinaksen@gmail.com |
Key Words |
Lysosomal; Gaucher; Niemann-Pick; Wolman; Cholesteryl ester; Children |
Core Tip |
Lysosomal storage disorders have a multisystem involvement. Gaucher disease, Niemann-Pick disease, and lysosomal acid lipase deficiency (Wolman disease and cholesteryl ester storage disorder) may have predominant liver dysfunction. Those with neonatal-onset, predominant severe extrahepatic and multi-systemic presentations often have challenging outcomes. Enzyme replacement therapy and liver transplantation are encouraged in select situations. Genetic tests and counseling are important aspects of management. |
Citation |
Sen Sarma M, Tripathi PR. Natural history and management of liver dysfunction in lysosomal storage disorders. World J Hepatol 2022; 14(10): 1844-1861 |
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Received |
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2021-12-27 08:20 |
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Peer-Review Started |
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2021-12-27 08:21 |
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To Make the First Decision |
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Return for Revision |
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2022-03-07 03:13 |
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Revised |
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2022-04-21 04:03 |
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Second Decision |
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2022-09-19 03:46 |
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Accepted by Journal Editor-in-Chief |
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Accepted by Company Editor-in-Chief |
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2022-09-21 21:43 |
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Articles in Press |
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2022-09-21 21:43 |
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Publication Fee Transferred |
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Edit the Manuscript by Language Editor |
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2022-10-21 05:31 |
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Typeset the Manuscript |
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2022-10-24 04:08 |
ISSN |
1948-5182 (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) 2022. Published by Baishideng Publishing Group Inc. All rights reserved. |
Permissions |
For details, please visit: http://www.wjgnet.com/bpg/gerinfo/207
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Publisher |
Baishideng Publishing Group Inc, 7041 Koll Center Parkway, Suite 160, Pleasanton, CA 94566, USA |
Website |
http://www.wjgnet.com |
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