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切迪阿克-希加希综合征与乳牙过早脱落

Chediak-Higashi syndrome and premature exfoliation of primary teeth.

作者信息

Rezende Karla Mayra, Canela Alfredo Hiram Carrillo, Ortega Adriana Oliveira Lira, Tintel Claudia, Bönecker Marcelo

机构信息

Department of Pediatric Dentistry, School of Dentistry, USP - University of São Paulo, São PauloSP, Brazil.

Department of Pediatric Dentistry, Universidad Autonoma de Asuncion, Asuncion, Paraguay.

出版信息

Braz Dent J. 2013 Nov-Dec;24(6):667-70. doi: 10.1590/0103-6440201302258.

Abstract

The Chediak-Higashi syndrome (CHS) is a rare hereditary fatal disease, if not treated. These changes are associated with various diseases and syndromes that mainly cause periodontal disease and thus the premature loss of teeth. This paper describes the monitoring of premature loss of primary teeth that began when the child was 5 years old. On presentation his teeth were mobile and there was a history of gingival bleeding. Panoramic radiography revealed generalized and severe bone loss, and the teeth showed no bony support enough for their stability. Blood test was ordered to assess the overall health of the child and giant cells with cytoplasmic granules were found, confirming the diagnosis of CHS. The management of periodontal disease focused on the control of infection and bacterial plaque by mechanical and chemical methods.

摘要

切迪阿克-希加希综合征(CHS)是一种罕见的遗传性致命疾病,若不治疗会导致死亡。这些变化与各种主要引发牙周病并进而导致牙齿过早脱落的疾病和综合征相关。本文描述了对一名5岁起就开始出现乳牙过早脱落情况的患儿的监测。初诊时,其牙齿松动,并有牙龈出血史。全景X线片显示广泛性严重骨质流失,牙齿没有足够的骨支持以维持稳固。进行了血液检查以评估患儿的整体健康状况,结果发现了含有细胞质颗粒的巨细胞,从而确诊为CHS。牙周病的治疗重点是通过机械和化学方法控制感染和牙菌斑。

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