Dental Evaluation of Kabuki Syndrome Patients
Dental Evaluation of Kabuki Syndrome Patients
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DOI：
10.1597/08-077.1
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发表时间：
2009-11-01
期刊：
影响因子：
--
通讯作者：
Kim, Chong Ae
中科院分区：
文献类型：
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作者：
Teixeira, Camila Santos;Leite Silva, Claudia Renata;Kim, Chong Ae
歌舞伎综合征是一种病因不明的遗传性疾病，其特征在于智力迟钝、生长缺陷和特殊的面部（即，睑裂长，下眼睑外侧三分之一外翻，耳朵突出，鼻尖宽而凹陷）。歌舞伎综合征中常见的口腔表现可能包括唇腭裂、双舌和悬雍垂、咬合不正和牙齿异常。我们评估了8例歌舞伎综合征患者的牙科表现。1例出现腭裂; 3例出现龋齿;有7人牙齿缺失，以上侧门齿和下中切牙缺失最为常见。所有缺失的牙齿都是永久性的，并且没有牙齿年代学或形态学的改变。由于大多数患者为混合牙列，因此通过父母的报告来评估乳牙的存在或缺失。1例患者出现上犬齿缺失，这在以前的文献中没有报道。牙科检查结果可能有助于临床诊断，或者它们可能是一个额外的发现，以证实诊断歌舞伎综合征的儿童轻度表型。
Kabuki syndrome is a genetic disorder of unknown etiology characterized by mental retardation, growth deficiency, and peculiar face (i.e., long palpebral fissures, eversion of the lateral third of the lower eyelids, prominent ears, and broad and depressed nasal tip). Oral manifestations commonly observed in Kabuki syndrome may comprise cleft lip/palate, bifid tongue and uvula, malocclusion, and dental abnormalities. We evaluated the dental findings of eight patients with Kabuki syndrome. One presented cleft palate; three presented caries; and seven had missing teeth, with the upper lateral incisors and inferior central incisors being the most commonly absent. All missing teeth were permanent, and there was no alteration of dental chronology or morphology. Because most patients had mixed dentition, the presence or absence of primary teeth was assessed through the parents' reports. One patient presented an absent upper canine, which had not been reported previously in the literature. Dental findings may be helpful for clinical diagnosis, or they may be an additional finding to substantiate the diagnosis of Kabuki syndrome in children with mild phenotype.