Delayed Eruption of Permanent Dentition and Maxillary Contraction in Patients with Cleidocranial Dysplasia: Review and Report of a Family.
Delayed Eruption of Permanent Dentition and Maxillary Contraction in Patients with Cleidocranial Dysplasia: Review and Report of a Family.
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DOI：
10.1155/2018/6591414
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发表时间：
2018
影响因子：
2.1
通讯作者：
Galluccio G
中科院分区：
文献类型：
--
作者：
Impellizzeri A;Midulla G;Romeo U;La Monaca C;Barbato E;Galluccio G
锁骨颅发育不良（CCD）是一种由6p21染色体RUNX2基因突变引起的遗传性疾病。这种常染色体显性或由自发基因突变引起的病理存在于百万分之一的个体中，具有完全外显性和广泛可变的表达性。通过PubMed界面识别2002 - 2015年文献报道中这些临床表现的发生率，并结合相关关键词。报告了当地患者的临床例子，与治疗方法有关。PubMed的研究结果是122篇文章。所有病例均报告了典型体征。94%的患者上颌骨发育不全。2例发现恒牙缺失：1例为II级颌缘，而非III级错颌。在我们的队列中也有类似的发现。CCD对牙科团队和患者来说都是一个挑战。治疗需要多学科的方法。需要进一步的研究来更好地了解这种疾病的原因。根据这篇综述，一个多步骤的方法提高了实现尽可能多的牙齿恢复的可能性，从而获得良好的咬合和更好的美学。
Cleidocranial dysplasia (CCD) is an inherited disease caused by mutations in the RUNX2 gene on chromosome 6p21. This pathology, autosomal dominant or caused by a spontaneous genetic mutation, is present in one in one million individuals, with complete penetrance and widely variable expressivity. To identify the incidence of these clinical findings in the report of the literature by means of PubMed interface from 2002 to 2015, with the related keywords. The report of local patients presents a clinical example, related to the therapeutic approach. The PubMed research resulted in 122 articles. All the typical signs were reported in all presented cases. The maxilla was hypoplastic in 94% of the patients. Missing of permanent teeth was found in two cases: one case presented a class II jaw relationship, instead of class III malocclusion. Similar findings were present in our cohort. CCD is challenging for both the dental team and the patient. The treatment requires a multidisciplinary approach. Further studies are required to better understand the cause of this disease. According to this review, a multistep approach enhances the possibilities to achieve the recovery of the most possible number of teeth, as such to obtain a good occlusion and a better aesthetic.