一例牙本质发育不良Ⅰ型患者的临床特点及其种植治疗

Clinical features and implant treatment in a patient with dentin dysplasia type Ⅰ

牙本质发育不良Ⅰ型(dentin dysplasia type Ⅰ,DD-Ⅰ)是一种伴有牙本质形成障碍、可遗传的罕见病。其特征是牙冠正常,但牙根发育异常,出现短、钝化和畸形的牙根;在患者年轻时即出现牙松动,可伴牙槽脓肿;影像学上表现为闭塞的牙髓腔。典型的DD-Ⅰ是常染色体显性遗传,家系中患者常很早即出现多颗牙丧失,有的30多岁即可表现为无牙颌。本文报告了一例DD-Ⅰ病例,通过总结其临床表现、影像学及组织学特点以及相关治疗,以期为DD-Ⅰ临床诊治提供指导。

Dentin dysplasia type Ⅰ(DD-Ⅰ)is a rare hereditary disturbance in dentin formation.It is characterized by the normal tooth crown but abnormal root development,which is manifested by the presence of short,blunted,and malformed roots;tooth loosening with alveolar abscess occurs at a young age in patients;imaging examination shows occluded pulp cavities.Typical dentin dysplasia type Ⅰ is autosomal dominant.Patients often loss multiple teeth,and may become edentulous by their 30s.Clinical treatment is challenging.In this paper,a DD-Ⅰ case is reported,summarizing its clinical manifestations,imaging and histological features,and related treatments in order to provide guidance for the clinical diagnosis and treatment of DD-Ⅰ.