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4/29/2025 9:24:19 AM | Browse: 24 | Download: 38
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Received |
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2025-01-27 08:16 |
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2025-01-27 08:18 |
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2025-03-18 21:07 |
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Revised |
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2025-03-31 18:53 |
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Second Decision |
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2025-04-21 02:36 |
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Accepted by Journal Editor-in-Chief |
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Accepted by Executive Editor-in-Chief |
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2025-04-21 05:23 |
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Articles in Press |
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2025-04-21 05:23 |
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Publication Fee Transferred |
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2025-04-01 12:29 |
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Edit the Manuscript by Language Editor |
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Typeset the Manuscript |
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2025-04-23 08:08 |
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Publish the Manuscript Online |
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2025-04-29 09:13 |
| ISSN |
1007-9327 (print) and 2219-2840 (online) |
| Open Access |
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: http://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 |
Case Report |
| Article Title |
Protein-losing enteropathy and multiple vasculature dysplasia in LZTR1-related Noonan syndrome: A case report and review of literature
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| Manuscript Source |
Unsolicited Manuscript |
| All Author List |
Qiu-Ju Tian, Lu-Jia Zhang, Qun Zhang, Feng-Chao Liu, Man Xie, Jin-Zhen Cai and Wei Rao |
| Funding Agency and Grant Number |
| Funding Agency |
Grant Number |
| Shandong Provincial Natural Science Fundation of China |
ZR2023QH015 |
| Qingdao Municipal Natural Science Fundation of China |
23-2-1-134-zyyd-jch |
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| Corresponding Author |
Wei Rao, MD, PhD, Professor, Division of Hepatology, Liver Disease Center, The Affiliated Hospital of Qingdao University, No. 59 Haier Road, Laoshan District, Qingdao 266100, Shandong Province, China. qdfy_raowei@qdu.edu.cn |
| Key Words |
Noonan syndrome; Leucine zipper-like transcription regulator 1; Protein-losing enteropathy; Porto-sinusoidal vascular disease; Hypoproteinemia; Intestinal lymphangiectasia; Case report |
| Core Tip |
Protein-losing enteropathy (PLE) is rarely reported with Noonan syndrome. This case reported a rare mutation in leucine zipper-like transcription regulator 1 related to Noonan syndrome, thoracic duct abnormalities, small intestinal lymphangiectasia, PLE in a juvenile female with multiple vasculature dysplasia and congenital deformity of the little finger, and the successful treatment of hypoalbuminemia by microsurgical relief of the thoracic duct outlet obstruction. As far as we know, this is the first case describing PLE associated with Noonan syndrome caused by a rare genetic mutation in leucine zipper-like transcription regulator 1 (c.850C>T:P.R284C) with newly reported manifestations. Additionally, this report provides new evidence about the management of Noonan syndrome. |
| Publish Date |
2025-04-29 09:13 |
| Citation |
<p>Tian QJ, Zhang LJ, Zhang Q, Liu FC, Xie M, Cai JZ, Rao W. Protein-losing enteropathy and multiple vasculature dysplasia in LZTR1-related Noonan syndrome: A case report and review of literature. <i>World J Gastroenterol</i> 2025; 31(17): 105347</p> |
| URL |
https://www.wjgnet.com/1007-9327/full/v31/i17/105347.htm |
| DOI |
https://dx.doi.org/10.3748/wjg.v31.i17.105347 |
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