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Articles Published Processes
2/21/2019 9:31:55 AM | Browse: 840 | Download: 1676
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Received |
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2018-10-22 06:26 |
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Peer-Review Started |
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2018-10-22 08:15 |
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To Make the First Decision |
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2018-12-12 07:25 |
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Return for Revision |
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2018-12-13 02:16 |
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Revised |
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2019-01-11 13:14 |
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Second Decision |
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2019-01-18 11:21 |
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Accepted by Journal Editor-in-Chief |
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Accepted by Executive Editor-in-Chief |
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2019-01-18 23:01 |
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Articles in Press |
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2019-01-18 23:01 |
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Publication Fee Transferred |
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Edit the Manuscript by Language Editor |
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2019-01-27 03:36 |
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Typeset the Manuscript |
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2019-02-20 10:24 |
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Publish the Manuscript Online |
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2019-02-21 09:31 |
| ISSN |
1007-9327 (print) and 2219-2840 (online) |
| Open Access |
This 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 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/ |
| Copyright |
© The Author(s) 2019. Published by Baishideng Publishing Group Inc. All rights reserved. |
| Article Reprints |
For details, please visit: http://www.wjgnet.com/bpg/gerinfo/247
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| 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 |
| Category |
Gastroenterology & Hepatology |
| Manuscript Type |
Retrospective Study |
| Article Title |
Δ4-3-oxosteroid-5β-reductase deficiency: Responses and long-term outcomes from oral bile acid therapy
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| Manuscript Source |
Unsolicited Manuscript |
| All Author List |
Mei-Hong Zhang, Kenneth DR Setchell, Jing Zhao, Jing-Yu Gong, Yi Lu and Jian-She Wang |
| ORCID |
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| Funding Agency and Grant Number |
| Funding Agency |
Grant Number |
| National Natural Science Foundation of China |
81570468 |
| Jinshan Science and Technology Commission |
2014-3-07 |
| National Natural Science Foundation of China |
81741056 |
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| Corresponding Author |
Jian-She Wang, MD, PhD, Professor, The Center for Pediatric Liver Diseases, Children's Hospital of Fudan University, 399 Wanyuan Road, Shanghai 201102, China. jshwang@shmu.edu.cn |
| Key Words |
Δ4-3-oxosteroid-5β-reductase deficiency; Mass spectrometry; Bile acid synthesis disorder; Chenodeoxycholic acid; Ursodeoxycholic acid |
| Core Tip |
Δ4-3-oxosteroid 5β-reductase (AKR1D1) deficiency presents as a particularly severe and rapidly progressive cholestasis. Treatment with oral primary bile acid has been shown to be effective in normalizing liver function circumventing the only alternative treatment of liver transplantation. The primary bile acid cholic acid is an approved drug for treating this genetic defect but is not available in China and many other countries. Here we report on the use of the alternative primary bile acid, chenodeoxycholic acid, in the largest cohort of patients with AKR1D1 deficiency studied to date, showing beneficial effects in a personalized regimen approach. |
| Publish Date |
2019-02-21 09:31 |
| Citation |
Zhang MH, Setchell KDR, Zhao J, Gong JY, Lu Y, Wang JS. Δ4-3-oxosteroid-5β-reductase deficiency: Responses and long-term outcomes from oral bile acid therapy. World J Gastroenterol 2019; 25(7): 859-869 |
| URL |
https://www.wjgnet.com/1007-9327/full/v25/i7/859.htm |
| DOI |
https://dx.doi.org/10.3748/wjg.v25.i7.859 |
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