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Copyright: ©Author(s) 2026. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution-NonCommercial (CC BY-NC 4.0) license. No commercial re-use. See permissions. Published by Baishideng Publishing Group Inc.
World J Diabetes. Oct 15, 2026; 17(10): 126085
Published online Oct 15, 2026. doi: 10.4239/wjd.126085
Maturity-onset diabetes of the young type 6 in China and the utility of semaglutide: A case report
Gui-Yan Chen, Liu-Xue Yang, Zhui Chen, Zhi-Qing Liang, Lin-Hua Pi
Gui-Yan Chen, Liu-Xue Yang, Zhui Chen, Lin-Hua Pi, Department of Endocrinology and Metabolism, The Second Affiliated Hospital of Guilin Medical University, Guilin 541004, Guangxi Zhuang Autonomous Region, China
Zhi-Qing Liang, Department of General Practice, The Second Affiliated Hospital of Guilin Medical University, Guilin 541004, Guangxi Zhuang Autonomous Region, China
Co-first authors: Gui-Yan Chen and Liu-Xue Yang.
Co-corresponding authors: Zhi-Qing Liang and Lin-Hua Pi.
Author contributions: Chen GY and Yang LX collected, analyzed and interpreted the patient data and was the major contributor to the writing of the manuscript, they contributed equally to this article, they are the co-first authors of this manuscript; Chen Z assisted with the patient data collection and analysis; Liang ZQ and Pi LH made substantial contributions to the data interpretation, manuscript revision, and conducted the work, they contributed equally to this article, they are the co-corresponding authors of this manuscript; and all authors are in agreement with the contents of the manuscript.
AI contribution statement: No AI tool was used to generate research data, interpret results, or formulate conclusions.
Supported by Guangxi Natural Science Foundation, No. 2026GXNSFAA00640444 and No. 2025GXNSFHA069136.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Corresponding author: Lin-Hua Pi, MD, PhD, Department of Endocrinology and Metabolism, The Second Affiliated Hospital of Guilin Medical University, No. 212 Renming Road, Guilin 541004, Guangxi Zhuang Autonomous Region, China. 18126970199@163.com
Received: July 24, 2026
Revised: August 6, 2026
Accepted: September 18, 2026
Published online: October 15, 2026
Processing time: 80 Days and 18.2 Hours
Abstract
BACKGROUND

Sulfonylureas and insulin constitute the standard therapy for patients with maturity-onset diabetes of the young 6 (MODY6) caused by mutation in neurogenic differentiation 1 gene but are characterized by an increased risk of hypoglycemia. Although glucagon-like peptide-1 receptor agonist (GLP-1 RA) may represent an effective treatment option, the utility of GLP-1 RA therapy in MODY6 has not been reported.

CASE SUMMARY

We report the case of a young man with MODY6 who was affected a mutation p.Asp331Glu in the neurogenic differentiation 1 gene and effectively and safely treated with semaglutide, which led to significant improvements in glycemic management and weight loss.

CONCLUSION

Our case report supports the classification of the p.Asp331Glu mutation as a likely pathogenicity of MODY6. And the use of GLP-1 RA under controlled conditions may represent an effective treatment option for patients with MODY6.

Keywords: Maturity-onset diabetes of the young; Neurogenic differentiation 1 gene; Glucagon-like peptide-1 receptor agonist; Semaglutide; Case report

Core Tip: Maturity-onset diabetes of the young type (MOD) 6, as a subtype of MODY caused by mutations in the neurogenic differentiation 1 gene, has been poorly reported, and its clinical features and treatments remain unclear. We report the case of a young man with MODY6 who was affected a mutation p.Asp331Glu in the neurogenic differentiation 1 gene and effectively and safely treated with semaglutide, which led to significant improvements in glycemic management and weight loss.

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