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9/25/2025 11:26:50 AM | Browse: 624 | Download: 471
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Received |
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2025-04-01 13:36 |
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Peer-Review Started |
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2025-04-01 13:37 |
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First Decision by Editorial Office Director |
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2025-05-01 02:26 |
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Return for Revision |
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2025-05-03 16:04 |
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Revised |
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2025-05-16 12:34 |
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Publication Fee Transferred |
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Second Decision by Editor |
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2025-08-07 02:44 |
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Second Decision by Editor-in-Chief |
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Final Decision by Editorial Office Director |
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2025-08-08 09:47 |
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Articles in Press |
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2025-08-08 09:47 |
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Edit the Manuscript by Language Editor |
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Typeset the Manuscript |
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2025-09-17 06:33 |
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Publish the Manuscript Online |
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2025-09-25 11:24 |
| 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) 2025. 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 |
Review |
| Article Title |
Pathogenesis and clinical management of liver damage in porphyrias: Mechanisms and therapeutic approaches
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| Manuscript Source |
Invited Manuscript |
| All Author List |
Tao Zeng, Shu-Ying Huang, Jian-Ning Chen, Jia-Hui Pang, Yu-Tian Chong and Xin-Hua Li |
| Funding Agency and Grant Number |
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| Corresponding Author |
Xin-Hua Li, Department of Infectious Diseases, Third Affiliated Hospital of Sun Yat-Sen University, No. 600 Tianhe Road, Guangzhou 510630, Guangdong Province, China, Guangzhou 510630, Guangdong Province, China. lixinh8@mail.sysu.edu.cn |
| Key Words |
Porphyria; Porphyria-related liver injury; Pathophysiology; Hepatic cutaneous porphyrias; Protoporphyria; Acute hepatic porphyrias |
| Core Tip |
Porphyria-related liver injury, driven by toxic accumulation of heme biosynthesis intermediates, presents diagnostic challenges due to nonspecific symptoms and insidious progression. The "Two-Hit Model" (porphyrin-protein aggregation and oxidative stress) underpins hepatotoxicity, while protoporphyria-associated cholestasis, liver failure, and acute hepatic porphyria-linked hepatocellular carcinoma (HCC) further increase morbidity. Diagnosis follows a multistep process integrating biochemical profiling, histopathological examination, and genetic testing. Current therapies are palliative, whereas emerging approaches—such as RNA interference (givosiran), melanocortin agonists (afamelanotide), and gene-editing technologies—provide mechanistic targeting. Early surveillance for HCC and multidisciplinary management are essential to mitigate life-threatening complications in these rare metabolic disorders. |
| Publish Date |
2025-09-25 11:24 |
| Citation |
Zeng T, Huang SY, Chen JN, Pang JH, Chong YT, Li XH. Pathogenesis and clinical management of liver damage in porphyrias: Mechanisms and therapeutic approaches. World J Hepatol 2025; 17(9): 107705 |
| URL |
https://www.wjgnet.com/1948-5182/full/v17/i9/107705.htm |
| DOI |
https://dx.doi.org/10.4254/wjh.v17.i9.107705 |
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