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下颌升支和冠突处的埋伏性第二恒磨牙:外胚层发育不良中WNT10A突变的一种新临床症状。

Impacted Lower Second Permanent Molars at the Ramus and Coronoid Process: A New Clinical Symptom of the WNT10A Mutation in Ectodermal Dysplasia.

作者信息

Sfeir Elia, Aboujaoude Samia

机构信息

Professor, Department of Pediatric Dentistry, School of Dentistry Lebanese University, Beirut, Lebanon.

Associate Professor, Department of Pediatric Dentistry, School of Dentistry Lebanese University, Beirut, Lebanon.

出版信息

Int J Clin Pediatr Dent. 2017 Oct-Dec;10(4):363-368. doi: 10.5005/jp-journals-10005-1466. Epub 2017 Feb 27.

Abstract

AIM

Hidrotic ectodermal dysplasia (ED) with the WNT10A mutation produces variable dentofacial symptoms. The aim of this study was to describe a new clinical symptom, i.e., specific to the WNT10A mutation in hidrotic ED. The study investigated the migratory trend of the lower second permanent molars to the ramus or coronoid process. To the best of authors' knowledge, no data in the literature describe this trend in cases of hidrotic ED.

MATERIALS AND METHODS

A three-generation family pedigree was established for seven families after the diagnosis of hidrotic ED in a 10-year-old boy. Thereafter, a genetic and clinical study was conducted on three families with at least one individual affected by hidrotic ED (20 individuals). We selected the children with molar germs 37 and 47. The eruption axes of these germs were then traced on the panoramic images at the initial time (T) and 1 year later (T + 1 year), and the deviations between these axes were measured.

RESULTS

A significant familial consanguinity was shown. Eight subjects presented with the hidrotic ED phenotype. Among them, three individuals carried germs 37 and 47. Over time, the measured deviations between the eruption axes of the latter displayed, in the majority of the cases, a distal inclination toward the ramus.

LIMITATIONS

A larger sample size is mandatory to assess the frequencies and treatment modalities.

CONCLUSION

The presence of germs in the lower second permanent molars in patients with hidrotic ED is an important clinical symptom that should be monitored to detect and prevent ectopic migration of these teeth.

CLINICAL SIGNIFICANCE

In hidrotic ED cases, the study of the presence of the second lower permanent germs must include clinical and radiological examinations. Establishing an inter-ceptive treatment is necessary to prevent the migration of the molars in question. Sfeir E, Aboujaoude S. Impacted Lower Second Permanent Molars at the Ramus and Coronoid Process: A New Clinical Symptom of the WNT10A Mutation in Ectodermal Dysplasia. Int J Clin Pediatr Dent 2017;10(4):363-368.

摘要

目的

伴有WNT10A突变的汗孔角化性外胚层发育不良(ED)会产生多种牙颌面症状。本研究的目的是描述一种新的临床症状,即汗孔角化性ED中WNT10A突变所特有的症状。该研究调查了下颌第二恒磨牙向升支或冠突的迁移趋势。据作者所知,文献中尚无关于汗孔角化性ED病例中这种趋势的数据。

材料与方法

在一名10岁男孩被诊断为汗孔角化性ED后,为7个家庭建立了三代家系图谱。此后,对至少有一名个体受汗孔角化性ED影响的3个家庭(20名个体)进行了遗传学和临床研究。我们选择了有37和47号磨牙牙胚的儿童。然后在初始时间(T)和1年后(T + 1年)的全景图像上追踪这些牙胚的萌出轴,并测量这些轴之间的偏差。

结果

显示出显著的家族近亲关系。8名受试者表现出汗孔角化性ED表型。其中,3名个体有37和47号牙胚。随着时间的推移,在大多数情况下,后者萌出轴之间测量的偏差显示出向升支的远中倾斜。

局限性

需要更大的样本量来评估频率和治疗方式。

结论

汗孔角化性ED患者下颌第二恒磨牙存在牙胚是一个重要的临床症状,应进行监测以检测和预防这些牙齿的异位迁移。

临床意义

在汗孔角化性ED病例中,对下颌第二恒牙胚存在情况的研究必须包括临床和放射学检查。建立预防性治疗对于防止相关磨牙的迁移是必要的。Sfeir E, Aboujaoude S. 位于升支和冠突的阻生下颌第二恒磨牙:外胚层发育不良中WNT10A突变的一种新临床症状。《国际临床儿科牙科学杂志》2017年;10(4):363 - 368。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c94e/5789140/c0361abc0300/ijcpd-10-363-g001.jpg

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