Division of Oral Infection and Disease Control, Department of Pediatric Dentistry, Osaka Graduate School of Dentistry, Suita, Osaka, Japan.
Department of Oral and Maxillofacial Radiology, Graduate School of Biomedical and Health Sciences, Hiroshima University, Hiroshima, Japan.
PLoS One. 2024 Jul 26;19(7):e0307896. doi: 10.1371/journal.pone.0307896. eCollection 2024.
X-linked hypophosphatemia (XLH) is the most common inherited form of rickets. The presence of sequence variations in the phosphate regulating endopeptidase homolog X-linked (PHEX) gene is associated with increased production of fibroblast growth factor 23 (FGF23). This results in renal phosphate wasting and impaired skeletal mineralization. Spontaneous dental abscesses, caused by endodontic infections resulting from hypomineralization of dentin, are a known dental complication of XLH. There is no objective method to evaluate the severity of dentin dysplasia. The purpose of this study was to develop a quantitative method to evaluate dentin dysplasia using orthopantomography that would allow the values in patients with XLH to be compared with the values in healthy participants of the same age.
The severity of dentin dysplasia was analyzed by measuring the pulp cavity area of the tooth using orthopantomographic images. The teeth analyzed were mandibular second primary molars and mandibular first permanent molars with complete root formation. Teeth with dental caries, restorations, or root resorption were excluded.
This retrospective observational study included a total of 200 images of healthy participants (aged 2-15 years) divided into five age groups and 42 images of 17 patients with XLH. There was a significant tendency for the pulp cavity area to decrease with increasing age in primary and permanent teeth. The pulp chambers of patients with XLH were larger than those of healthy participants in primary and permanent teeth.
We have established a method of using orthopantomography for quantitative assessment of dentin dysplasia in XLH from the primary dentition to the permanent dentition. Evaluating the severity of dentin hypomineralization by this method is useful in the diagnosis of the dental manifestations of XLH. Early diagnosis of XLH enables oral management and leads to prevention of dental abscesses.
X 连锁低磷血症（XLH）是最常见的遗传性佝偻病形式。磷酸调节内肽酶同源物 X 连锁（PHEX）基因的序列变异与成纤维细胞生长因子 23（FGF23）的产量增加有关。这导致肾脏磷酸盐丢失和骨骼矿化受损。由于牙本质矿化不足导致的牙髓感染引起的自发性牙脓肿是 XLH 的已知牙科并发症。目前还没有客观的方法来评估牙本质发育不良的严重程度。本研究旨在开发一种使用全景片评估牙本质发育不良的定量方法，以便将 XLH 患者的值与同年龄健康参与者的值进行比较。
通过使用全景片图像测量牙齿的牙髓腔面积来分析牙本质发育不良的严重程度。分析的牙齿为下颌第二乳磨牙和下颌第一恒磨牙，且根完全形成。排除有龋齿、修复体或根吸收的牙齿。
本回顾性观察研究共纳入了 200 张健康参与者（年龄 2-15 岁）的全景片，分为 5 个年龄组，以及 42 张 17 名 XLH 患者的全景片。在乳恒牙中，牙髓腔面积随年龄的增加而呈显著下降趋势。XLH 患者的牙髓腔在乳恒牙中均大于健康参与者。
我们已经建立了一种使用全景片从乳牙到恒牙定量评估 XLH 牙本质发育不良的方法。通过这种方法评估牙本质矿化不足的严重程度有助于 XLH 牙科表现的诊断。早期诊断 XLH 可以进行口腔管理，从而预防牙脓肿。
