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一例早老症(哈钦森-吉尔福德综合征)的口腔颌面外科考量

Oral and maxillofacial surgical considerations for a case of Hutchinson-Gilford progeria.

作者信息

Batstone M D, Macleod A W G

机构信息

University of Queensland, Australia.

出版信息

Int J Paediatr Dent. 2002 Nov;12(6):429-32. doi: 10.1046/j.1365-263x.2002.00401.x.

DOI:10.1046/j.1365-263x.2002.00401.x
PMID:12452985
Abstract

Hutchinson-Guilford progeria is a rare genetic condition showing the stigmata of accelerated ageing combined with severe growth retardation. Patients with this condition show a classical facies and clinical features with an average age of death of 13, usually due to atherosclerotic changes. Craniofacial and dental manifestations include mandibular and maxillary hypoplasia, both vertically and horizontally. Delayed and abnormal tooth eruption and morphology are commonly present. The long-term medical prognosis and eruption potential of individual teeth is important when considering treatment. In addition to this, surgical planning and surgical technique must be modified by the abnormal facial morphology, dermal inelasticity, potential anaesthetic difficulties, and ongoing deterioration in the medical condition. These factors mandate early and definitive intervention for oral surgical conditions. We report the case of a 13-year-old male treated for pericoronitis and oral pain relating to delayed eruption of first permanent molars.

摘要

哈钦森-吉尔福德早衰症是一种罕见的遗传疾病,表现出加速衰老的特征并伴有严重生长发育迟缓。患有这种疾病的患者呈现出典型面容和临床特征,平均死亡年龄为13岁,通常死于动脉粥样硬化改变。颅面和牙齿表现包括下颌骨和上颌骨垂直及水平方向发育不全。牙齿萌出延迟和异常以及形态异常普遍存在。在考虑治疗时,单个牙齿的长期医学预后和萌出潜力很重要。除此之外,手术规划和手术技术必须根据异常的面部形态、皮肤弹性减退、潜在的麻醉困难以及病情的持续恶化进行调整。这些因素要求对口腔外科疾病进行早期和确定性干预。我们报告一例13岁男性患者,因第一恒磨牙萌出延迟导致冠周炎和口腔疼痛而接受治疗。

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An infant with congenital micrognathia and upper airway obstruction was diagnosed as Hutchinson-Gilford progeria syndrome caused by a novel LMNA mutation: Case report and literature review.一名患有先天性小颌畸形和上呼吸道梗阻的婴儿被诊断为由一种新的LMNA突变引起的哈钦森-吉尔福德早衰综合征:病例报告及文献综述。
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