国际口腔医学杂志

国际口腔医学杂志 ›› 2022, Vol. 49 ›› Issue (3): 332-336.doi: 10.7518/gjkq.2022030

• 综述 • 上一篇    下一篇

牙本质发育不良Ⅰ型及其分型治疗

雷彬1(),陈柯2()   

  1. 1.暨南大学口腔医学院 广州 510000
    2.南方医科大学口腔医院海珠广场院区儿童口腔科 广州 510000
  • 收稿日期:2021-07-21 修回日期:2021-11-06 出版日期:2022-05-01 发布日期:2022-05-09
  • 通讯作者: 陈柯
  • 作者简介:雷彬，住院医师，硕士，Email：435186977@qq.com
  • 基金资助:
    南方医科大学口腔医院高层次人才项目(PY2018022)

Classification and treatment of dentin dysplasia type Ⅰ

Lei Bin1(),Chen Ke2()   

  1. 1.School of Stomatology, Jinan University, Guangzhou 510000, China
    2.Dept. of Pediatric Dentistry, Haizhu Square, Stomatological Hospital of Southern Medical University, Guangzhou 510000, China
  • Received:2021-07-21 Revised:2021-11-06 Online:2022-05-01 Published:2022-05-09
  • Contact: Ke Chen
  • Supported by:
    High-level Talents Project of Stomatological Hospital, Southern Medical University(PY2018022)

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摘要：

牙本质发育不良Ⅰ型（DD-Ⅰ）是一种罕见的遗传性牙本质形成障碍疾病，乳恒牙均可受累。该病在临床上表现为牙冠外形色泽正常，牙齿松动明显，可伴有自发性牙槽脓肿或囊肿等。影像学检查则可见牙髓腔消失或呈“新月形”牙髓残余，根短钝或无牙根等表现。关于DD-Ⅰ的发病机制已为大多数学者所研究，其临床治疗通常具有挑战性，本文对近年来DD-Ⅰ的临床分型及表现、致病基因、组织学特点、治疗相关研究进行综述，以期为临床诊治该病提供一定的指导。

关键词: 牙本质发育不良Ⅰ型, 分型, 发病机制, 致病基因, 治疗

Abstract:

Dentin dysplasia type Ⅰ (DD-Ⅰ) is a rare genetic disorder of dentine formation that affects all deciduous and permanent teeth. In this disease, the tooth crown’s appearance, color, and lustre are normal; tooth loosening is obvious; and spontaneous tooth alveolar abscess or cyst may be present. Imaging examinations show that the pulp cavity disappeared or the residual pulp has a “crescent shape” and the root is short and blunt or the root is absent. The pathogenesis of DD-Ⅰ has been studied by most scholars, and its clinical treatment is often challenging. In this paper, the clinical classification and manifestations, pathogenic genes, histological characteristics, and treatment of DD-Ⅰ in recent years are reviewed to provide guidance for the clinical diagnosis and treatment of this disease.

Key words: dentine dysplasia type Ⅰ, classification, pathogenesis, pathogenic gene, treatment

中图分类号: 

  • R 781.2

图 1

DD-Ⅰ亚分类Ⅰa型：髓腔完全闭塞，无牙根形成；Ⅰb型：单条水平新月形牙髓残留，牙根极短，无根管影像；Ⅰc型：2条水平新月形的牙髓残留，牙根发育不完整，无根管影像；Ⅰd型：髓腔及根管冠部可见椭圆形的牙髓结石，牙根长度正常。"

表 1

遗传性牙本质疾病的致病基因"

疾病类型基因位置致病基因
DGI-Ⅰ17q21.33COL1A1
7q21.3COL1A2
DGI-Ⅱ4q22.1DSPP
DGI-Ⅲ4q22.1DSPP
DD-Ⅰ6q27SMOC2
18q21.33VPS4B
3p26.1SSUH2
DD-Ⅱ4q22.1DSPP
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