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《9 岁男孩黏多糖贮积症：口腔牙科管理和诊断注意事项》：病例报告。

"Mucopolysaccharidosis syndrome in a 9-Year-old boy: oral-dental management and diagnostic considerations": a case report.

机构信息

Department of Pediatrics, Dental Research Center, Dental Research Institute, School of Dentistry, Isfahan University of Medical Sciences, Isfahan, Iran.

Department of Pediatrics, School of Dentistry, Isfahan University of Medical Sciences, Isfahan, Iran.

出版信息

BMC Oral Health. 2024 Sep 27;24(1):1140. doi: 10.1186/s12903-024-04859-6.

DOI:10.1186/s12903-024-04859-6
PMID:39334095
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11438088/
Abstract

BACKGROUND

Mucopolysaccharidosis (MPS) comprises a group of metabolic diseases with a disorder in the function of lysosomal enzymes that lead to the accumulation of glycosaminoglycans (mucopolysaccharides) (Kubaski et al. in Diagnostics 10:161, 2020; Hampe et al. in Cells 9:1838, 2020; Tomatsu et al. Mol Genet Metab 110(1-2):42-53, 2013). At least seven variants of this disorder have been identified to date (Muenzer et al. in Pediatrics 124(6):e1228-e1239, 2009; Muenzer et al. in Eur J Pediatr 171:181-8, 2012). this study aims to report a case of mucopolysaccharidosis in a 9-year-old child. Also, the treatments and dental observations made for the child have been described. Also, a review of past articles has been done to report the types of diseases, medical and dental considerations, etc. of this disease.

CASE DESCRIPTION

the present case report describes the orofacial and systemic characteristics, diagnostic methods, and dental management of a 9-year-old boy with MPS with a one-year follow-up in association with a brief review of past articles.

CONCLUSION

Since MPS patients have many changes in their oral and dental structures, they pose many challenges for dentists. Also, these patients need considerations in dentistry due to the involvement of different body organs. Knowing the oral-dental and systemic problems of these patients can help their dentists to provide effective and safe treatment for them.

摘要

背景

黏多糖贮积症（MPS）是一组代谢疾病，其溶酶体酶功能紊乱导致糖胺聚糖（黏多糖）积累（Kubaski 等人，2020 年；Hampe 等人，2020 年；Tomatsu 等人，2013 年）。迄今为止，已经确定了至少七种这种疾病的变体（Muenzer 等人，2009 年；Muenzer 等人，2012 年）。本研究旨在报告一例 9 岁儿童的黏多糖贮积症病例，并描述了对该儿童进行的治疗和牙科观察。此外，还对过去的文章进行了综述，以报告该疾病的疾病类型、医学和牙科注意事项等。

病例描述

本病例报告描述了一名 9 岁 MPS 男孩的口颌系统特征、诊断方法和牙科管理情况，并结合过去文章的简要回顾进行了随访一年。

结论

由于 MPS 患者的口腔和牙齿结构有许多变化，因此给牙医带来了许多挑战。此外，由于涉及不同的身体器官，这些患者在牙科方面也需要考虑。了解这些患者的口腔-牙齿和全身问题，可以帮助他们的牙医为他们提供有效和安全的治疗。

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The importance of skeletal x-ray screening for dysostosis multiplex in the early diagnosis of mucopolysaccharidosis.骨骼X线筛查在黏多糖贮积症早期诊断中对多发性骨发育异常的重要性。
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