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家族性非综合征型多发性额外牙伴正常核型:病例报告。

Non-syndromic multiple supernumerary teeth in a family unit with a normal karyotype: case report.

机构信息

Department of Dental Sciences and Surgery, University of Bari, Bari, Italy.

出版信息

Int J Med Sci. 2010 Nov 5;7(6):378-84. doi: 10.7150/ijms.7.378.

Abstract

Introduction. Hyperdontia is an odontostomatologic anomaly characterized by an excess in tooth number. It seems to occur more often in patients with hereditary factors concerning this anomaly: this case represents a rare form of hyperdontia, with bilateral multiple supernumerary teeth, with evident penetrance of the phenotype in the family unit engaged in the present study. The karyotype determination excludes a pathogenesis on chromosomal basis.Case report. A 30 years old patient came to our observation with five impacted teeth (1.8, 2.8, 3.8, 4.7 and 4.8), as well as with the presence of an impacted supernumerary tooth (distomolar 4.9). The patient was suggested to allow us to perform a radiologic screening to his two sisters aged 17 and 13 years.The X-ray photography showed that the elder sister had nine impacted teeth; these were 1.8 - 1.9 - 2.8 - 2.9 - 2.10 - 3.8 - 3.9 - 4.8 - 4.9; while the youngest sister had four impacted teeth, that is 1.8 - 1.9 - 2.8 - 2.9.Conclusions. The value of the present case report can be used as a paradigm for the assessment of the hereditary factors predisposing the onset of hyperdontia, and for the consequent management by oral surgeon of family units in which the odontostomatologic anomaly was detected without any syndromic forms.

摘要

简介。多生牙是一种牙源性口腔异常,其特征是牙齿数量过多。它似乎更多地发生在具有与该异常相关的遗传因素的患者中:这种情况代表了一种罕见的多生牙形式,具有双侧多发性额外牙,在参与本研究的家族单位中表现出明显的表型外显率。染色体核型分析排除了基于染色体的发病机制。病例报告。一名 30 岁患者前来就诊,其有五颗阻生牙(1.8、2.8、3.8、4.7 和 4.8),还有一颗阻生额外牙(4.9 远中磨牙)。建议患者允许我们对其两名年龄分别为 17 岁和 13 岁的姐妹进行影像学筛查。X 射线摄影显示,姐姐有九颗阻生牙:1.8-1.9-2.8-2.9-2.10-3.8-3.9-4.8-4.9;而妹妹有四颗阻生牙,即 1.8-1.9-2.8-2.9。结论。本病例报告的价值可作为评估易患多生牙的遗传因素的范例,以及口腔外科医生对未出现任何综合征形式的牙源性口腔异常的家族单位进行后续管理的范例。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0552/2974166/6d2c6268efbe/ijmsv07p0378g01.jpg

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本文引用的文献

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