描绘与交界型大疱性表皮松解症相关的综合征型釉质发育不全中的外部牙冠吸收
Depicting External Crown Resorption in Syndromic Amelogenesis Imperfecta Associated With Junctional Epidermolysis Bullosa.
文献信息
| PMID | 42956167 |
|---|---|
| 原文 | 在 PubMed 查看原文 ↗ |
| 发表日期 | 2026 |
| 作者 | Susanne Krämer |
| 作者单位 | Department of Dermatology, Medical Faculty and Medical Centre, University of Freiburg, Freiburg, Germany. |
| 期刊 | Clinical and experimental dental research |
| SCI 分区 | Q2 |
| IF | 2.5 |
| 研究类型 | 临床研究 · 临床 |
| 所属专科 | 鼻科 |
中文摘要
目的： 描述与交界型大疱性表皮松解症（JEB）相关的综合征型釉质发育不全（SAI）患者中的外部牙冠吸收（ECR）。
材料与方法： 在两家中心（德国和智利）开展了一项多中心回顾性研究。分析的变量包括牙列、牙齿、牙面、年龄、严重程度、进展、治疗策略和临床结局。
结果： 共纳入来自30例患者的170颗受ECR影响的牙齿（60颗乳牙，110颗恒牙）。患者平均有5.7±4.0颗受累牙齿。在该队列中，乳牙列中切牙占受累牙齿的88%，尖牙占8%，磨牙占3%。恒牙列中，62%为切牙，19%为磨牙，10%为尖牙，9%为前磨牙。8颗牙齿显示两个独立的起始部位，在170颗牙齿中形成178个受累牙面；其中85.4%的牙面为切端/咬合面，12.9%为邻面，1.7%为前庭/腭侧。检出年龄在乳牙列为8个月至8.7岁，在恒牙列为5.2至22岁。严重程度分析显示，后牙中严重受损磨牙的比例更高（61.9%），而前牙中中度（恒牙57.4%）和轻度（乳牙37.7%）受损的比例更高。基于39颗牙齿观察到的进展间隔为4个月至9.3年。文中介绍了影像学研究、治疗策略及其结局。与仅采用预防策略相比，修复治疗与进一步结构丧失的频率较低相关。提出了一项暂行的放射学筛查方案。
结论： ECR可影响乳牙列和恒牙列，若不进行修复可导致牙齿丧失。本文提出的用于ECR早期诊断和治疗的放射学筛查方案有助于保留牙齿。
英文摘要
OBJECTIVES: To characterize External Crown Resorption (ECR) in patients with Syndromic Amelogenesis Imperfecta (SAI) associated with junctional epidermolysis bullosa (JEB).
MATERIALS AND METHODS: A multicentre retrospective study was conducted at two centers (Germany and Chile). Analyzed variables included dentition, tooth, surface, age, severity, progression, treatment strategy, and clinical outcome.
RESULTS: One hundred and seventy teeth affected with ECR (60 primary, 110 permanent) from 30 patients were included. Patients presented an average of 5.7 ± 4.0 affected teeth. Within this cohort, in primary dentition, incisors represented 88% of affected teeth, 8% canines, and 3% molars. In permanent dentition, 62% were incisors, 19% molars, 10% canines, and 9% premolars. Eight teeth showed two independent initiation sites, giving 178 affected surfaces in 170 teeth; 85.4% of these surfaces were incisal/occlusal, 12.9% proximal, and 1.7% vestibular/palatal. Age of detection varied from 8 months to 8.7 years (primary dentition) and from 5.2 to 22 years in permanent dentition. Severity analysis revealed that posterior teeth presented a higher proportion of severely compromised molars (61.9%), while anterior teeth presented a higher proportion of more moderate (57.4% permanent) and mild (37.7% primary) compromise. Observed progression intervals, based on 39 teeth, ranged from 4 months to 9.3 years. Imaging studies, treatment strategies, and their outcomes are presented. Restoration was associated with a lower frequency of further structural loss than preventive strategies alone. A provisional radiographic screening protocol is proposed.
CONCLUSION: ECR can affect primary and permanent dentition, leading to tooth loss if left unrestored. The radiographic screening protocol for early diagnosis and treatment of ECR proposed in this article can aid in preserving teeth.