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桑贾德 - 萨卡蒂综合征的牙颌面特征:一例报告

The dentofacial features of Sanjad-Sakati syndrome: a case report.

作者信息

Al-Malik M I

机构信息

Dental Department, King Fahd Armed Forces Hospital, PO Box 419, Jeddah 21411, Saudi Arabia.

出版信息

Int J Paediatr Dent. 2004 Mar;14(2):136-40. doi: 10.1111/j.1365-263x.2004.00527.x.

Abstract

Sanjad-Sakati syndrome is an autosomal recessive disorder that was first reported by Sanjad et al. in 1988. It is characterized by congenital hypoparathyroidism, severe growth failure and dysmorphic features. The clinical features include deep set eyes, microcephaly, thin lips, depressed nasal bridge with beaked nose, external ear anomalies and learning difficulties. This report presents the case of a 4-year-old patient who was referred to our dental clinic because of pain in her mouth and poor dental health. Oral findings included micrognathic mandible and maxilla, microdontia, enamel hypoplasia as well as severely decayed teeth. Treatment was carried out under general anaesthesia to extract the most severely affected teeth and restore those which could be conserved. It was concluded that these patients have special dental needs; early diagnosis of the affected children is therefore important in order to commence preventive dental therapy and carry out appropriate dental treatment at the optimum time.

摘要

桑贾德 - 萨卡蒂综合征是一种常染色体隐性疾病,于1988年由桑贾德等人首次报道。其特征为先天性甲状旁腺功能减退、严重生长发育迟缓及畸形特征。临床特征包括深陷的眼睛、小头畸形、薄唇、鼻梁凹陷伴鹰钩鼻、外耳异常及学习困难。本报告介绍了一名4岁患者的病例,该患者因口腔疼痛和口腔健康不佳转诊至我们的牙科诊所。口腔检查发现包括下颌骨和上颌骨发育不全、牙齿过小、釉质发育不全以及严重龋齿。在全身麻醉下进行治疗,拔除受影响最严重的牙齿,并修复那些可以保留的牙齿。得出的结论是,这些患者有特殊的口腔需求;因此,对患病儿童进行早期诊断很重要,以便开始预防性口腔治疗并在最佳时机进行适当的口腔治疗。

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