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非综合征性牙齿缺失患者的治疗策略:病例报告及文献复习。

Treatment strategy for patient with non-syndromic tooth agenesis: a case report and literature review.

机构信息

Department of Stomatology, Nanfang Hospital, Southern Medical University, 1838 N Guangzhou RD, Guangzhou, 510080, Guangdong, People's Republic of China.

出版信息

BMC Oral Health. 2024 Jul 25;24(1):840. doi: 10.1186/s12903-024-04613-y.

DOI:10.1186/s12903-024-04613-y
PMID:39048976
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11270777/
Abstract

BACKGROUND

Non-syndromic tooth agenesis (NSTA) is a type of ectodermal dysplasia (ED) in which patients with non-syndromic oligodontia may only affect teeth. No pathological findings were found in other tissues of the ectodermal. Herein, we report a case of a NSTA patient with severe dental anxiety and poor oral health.

CASE PRESENTATION

A 5-year-old boy without systemic diseases presented as a patient with oligodontia, extensive caries, and periapical periodontitis. Molecular genetic analysis found a mutation in the Ectodysplasin A (EDA) gene, confirming the diagnosis of NSTA.

CONCLUSION

Tooth agenesis (TA) is the most common ectodermal developmental abnormality in humans. Non-syndromic oligodontia patients often seek treatment in the department of stomatology. Because of their complex oral conditions, these patients should be provided with a systematic and personalized treatment plan.

摘要

背景

非综合征性牙齿缺失(NSTA)是一种外胚层发育不良(ED),其中非综合征性少牙症患者可能仅影响牙齿。在外胚层的其他组织中未发现病理发现。在此,我们报告了一例 NSTA 患者,其患有严重的牙齿焦虑和口腔健康不良。

病例介绍

一名 5 岁男孩无系统疾病,表现为少牙症、广泛龋齿和根尖周炎。分子遗传学分析发现 Ectodysplasin A(EDA)基因突变,确诊为 NSTA。

结论

牙齿缺失(TA)是人类最常见的外胚层发育异常。非综合征性少牙症患者通常在口腔科就诊。由于其复杂的口腔状况,应针对这些患者提供系统和个性化的治疗计划。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6b7c/11270777/a08cfcc43d5d/12903_2024_4613_Fig5_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6b7c/11270777/c7ed668ed2fc/12903_2024_4613_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6b7c/11270777/22b2645bb303/12903_2024_4613_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6b7c/11270777/a3b8e3797349/12903_2024_4613_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6b7c/11270777/7c94c169b670/12903_2024_4613_Fig4_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6b7c/11270777/a08cfcc43d5d/12903_2024_4613_Fig5_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6b7c/11270777/c7ed668ed2fc/12903_2024_4613_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6b7c/11270777/22b2645bb303/12903_2024_4613_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6b7c/11270777/a3b8e3797349/12903_2024_4613_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6b7c/11270777/7c94c169b670/12903_2024_4613_Fig4_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6b7c/11270777/a08cfcc43d5d/12903_2024_4613_Fig5_HTML.jpg

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本文引用的文献

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Congenital Tooth Agenesis and Risk of Early-Onset Cancer.先天性牙齿缺失与早发性癌症风险。
JAMA Netw Open. 2024 Mar 4;7(3):e240365. doi: 10.1001/jamanetworkopen.2024.0365.
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Rare Pediatric Genetic Case Report of X-linked Hypohidrotic Ectodermal Dysplasia Type 1.1型X连锁低汗性外胚层发育不良的罕见儿科遗传病例报告
Cureus. 2023 Dec 2;15(12):e49840. doi: 10.7759/cureus.49840. eCollection 2023 Dec.
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Rehabilitation with implant-supported overdentures in preteens patients with ectodermal dysplasia: A cohort study.青少年外胚层发育不良患者使用种植体支持覆盖义齿的康复治疗:一项队列研究。
Clin Implant Dent Relat Res. 2023 Dec;25(6):1187-1196. doi: 10.1111/cid.13258. Epub 2023 Aug 22.
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The EDA/EDAR/NF-κB pathway in non-syndromic tooth agenesis: A genetic perspective.非综合征性牙齿发育不全中的EDA/EDAR/NF-κB信号通路:遗传学视角
Front Genet. 2023 Apr 3;14:1168538. doi: 10.3389/fgene.2023.1168538. eCollection 2023.
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Expanding the genetic spectrum of tooth agenesis using whole-exome sequencing.全外显子测序拓展牙齿缺失症的遗传谱。
Clin Genet. 2022 Dec;102(6):503-516. doi: 10.1111/cge.14225. Epub 2022 Sep 17.
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Rehabilitation Considerations for Very Young Children with Severe Oligodontia due to Ectodermal Dysplasia: Report of Three Clinical Cases with a 2-Year Follow-Up.外胚层发育不良所致严重少牙畸形幼儿的康复考量:三例临床病例报告及两年随访
Case Rep Dent. 2022 Mar 22;2022:9925475. doi: 10.1155/2022/9925475. eCollection 2022.
7
The long-term effect of dental treatment under general anaesthesia or physical restraints on children's dental anxiety and behaviour.在全身麻醉或身体约束下进行牙科治疗对儿童牙科焦虑和行为的长期影响。
Eur J Paediatr Dent. 2022 Mar;23(1):27-32. doi: 10.23804/ejpd.2022.23.01.05.
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Clinical outcomes of dental treatment under general anesthesia and its effects on the caries activity and body growth of children: a 2-year retrospective study.全麻下口腔治疗的临床效果及其对儿童龋齿活动度和身体生长的影响:一项 2 年回顾性研究。
Clin Oral Investig. 2022 May;26(5):4091-4098. doi: 10.1007/s00784-022-04377-1. Epub 2022 Feb 3.
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Hua Xi Kou Qiang Yi Xue Za Zhi. 2021 Dec 1;39(6):703-708. doi: 10.7518/hxkq.2021.06.012.