Craniofacial and dental phenotype of Smith-Magenis syndrome.
Craniofacial and dental phenotype of Smith-Magenis syndrome.
复制标题
史密斯-马吉尼斯综合征的颅面和牙齿表型。
DOI：
10.1002/ajmg.a.31371
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发表时间：
2006
期刊：
影响因子：
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通讯作者：
Hart,ThomasC
中科院分区：
文献类型：
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作者：
Tomona,Natalia;Smith,AnnCM;Guadagnini,JeanPierre;Hart,ThomasC
这项研究的目的是评估和描述确诊为Smith-Magenis综合征(SMS)的个体的牙科和颅面部表现。对15例4~19岁儿童行口外及口内检查，包括牙位片、头面部X线片和面部三维摄影。主要累及下颌第二前磨牙的牙齿发育不良(13/15例)和牛根牙畸形(13/15例)是常见的临床表现。三分之一的病例有牙根扩张。每个病例中至少有一个牙齿异常。这些发现发生的频率比一般人群更高(P<； 0.001)。与年龄相关的龋齿和修复牙的增加被发现。口腔卫生较差，牙菌斑增多，牙周炎加重，从儿童到青少年时期都有进展。放射学检查结果表明，下颌骨过度生长并不是导致颌骨突出的原因。其他发现包括下颌前牙前突、骨下巴增大和巨舌症，这可能是导致下颌前牙突出的原因。牙齿异常的高患病率(>；90%)进一步扩大了表型，表明牙齿评估可能有助于诊断多发性硬化症。2006年出版，Wiley-Liss，Inc.
The aim of this study was to assess and characterize dental and craniofacial findings in individuals with a confirmed diagnosis of Smith–Magenis syndrome (SMS). Extraoral and intraoral examination including dental and craniofacial radiographs and three‐dimensional facial photoimaging were performed for 15 cases between ages 4 and 19 years old. Tooth agenesis (13/15 cases) affecting primarily the mandibular second premolars and taurodontism (13/15 cases) were common findings. Dilaceration of the tooth roots was present in one‐third of the cases. At least one dental anomaly was present in each case. These findings occur with greater frequency than in the general population (P< 0.001). An age‐related increase in decayed and restored teeth was found. Poorer oral hygiene, increased dental plaque, and increased gingival inflammation progressed from childhood to teenage years. Radiographic findings suggest the prognathic appearance is not caused by excessive mandibular growth. Other findings including protrusion of the mandibular anterior teeth, increased bony chin size, and macroglossia were noted, which may contribute to the prognathic appearance. The high prevalence of dental anomalies (>90%) further expands the phenotype and indicates that dental evaluation may aid in the diagnosis of SMS. Published 2006 Wiley‐Liss, Inc.