Characteristics of oral health of patients with X-linked hypophosphatemia: case reports and literature review.

IF 2.5 Q2 DENTISTRY, ORAL SURGERY & MEDICINE BDJ Open Pub Date : 2024-05-31 DOI:10.1038/s41405-024-00223-6
Ana Arhar, Alenka Pavlič, Luka Hočevar
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Abstract

Background: Oral health is impaired in X-linked hypophosphatemia (XLH), resulting in delayed dental development, malocclusion, and radiographic abnormalities. This study investigates the oral manifestations in Slovenian XLH patients, focusing on enamel and dentin abnormalities and a literature review of spontaneous periapical abscesses in XLH cases.

Objectives: To report XLH patients with specific oral signs and symptoms, histological analysis of affected teeth, and review of reported cases of XLH patients with spontaneous periapical abscesses.

Methods: Case reports: Seven XLH patients from the National Registry of Patients with Rare Diseases underwent a detailed oral examination, including X-ray reviews. The patients who were expected to have tooth exfoliation or extraction were asked to donate their teeth for histological analysis by scanning electron microscopy.

Literature search: A literature search of four electronic databases and a manual bibliography search aimed to identify documented cases of XLH with periapical abscesses up to January 21, 2024. Inclusion criteria were confirmed XLH patients with periapical abscesses in English peer-reviewed publications.

Results: Tooth samples from three XLH patients showed reduced dentin mineralisation, affecting one-third to one-half of the outer dentin. Inadequate mineralisation, uneven dentin tubules, and cracks and chipping in the enamel were observed, indicating mineralisation deviations. Similar cracks extended into the dentin and were also present in the root of the examined tooth. Based on the content of the 75 items identified in the search, spontaneous abscesses are not uncommon in patients with XLH.

Conclusions: XLH significantly affects patients' lives and requires lifelong treatment. Dental examinations consistently revealed oral problems, including malocclusion. Histological analysis confirmed structural changes, especially in the dentin. Despite continued treatment, XLH patients may have an increased risk of oral pathologies. Further research is needed to understand the impact of XLH and its treatment on dental health.

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X 连锁低磷血症患者口腔健康的特点:病例报告和文献综述。
背景:X连锁低磷血症(XLH)患者的口腔健康受损,导致牙齿发育迟缓、咬合不正和影像学异常。本研究调查了斯洛文尼亚 XLH 患者的口腔表现,重点关注牙釉质和牙本质异常,并对 XLH 病例中自发性根尖周炎的文献进行了回顾:报告有特殊口腔症状和体征的 XLH 患者,对受影响牙齿进行组织学分析,并回顾自发性根尖周脓肿 XLH 患者的报告病例:病例报告:来自国家罕见病患者登记处的七名 XLH 患者接受了详细的口腔检查,包括 X 光检查。预计会出现牙齿脱落或拔除的患者被要求捐献牙齿,以便通过扫描电子显微镜进行组织学分析:文献检索:对四个电子数据库进行了文献检索,并对书目进行了人工检索,目的是找出截至 2024 年 1 月 21 日有记录的伴有根尖周脓肿的 XLH 病例。纳入标准为经同行评审的英文出版物中证实的XLH患者根尖周脓肿病例:三名 XLH 患者的牙齿样本显示牙本质矿化度降低,三分之一到二分之一的外层牙本质受到影响。矿化不足、牙本质小管不均匀、珐琅质出现裂缝和碎裂,表明矿化出现偏差。类似的裂纹延伸至牙本质,在受检牙齿的根部也有出现。根据搜索到的 75 项内容,XLH 患者自发性脓肿并不少见:结论:XLH 严重影响患者的生活,需要终生治疗。牙科检查一致发现口腔问题,包括咬合不正。组织学分析证实了结构的变化,尤其是牙本质的变化。尽管继续接受治疗,XLH 患者出现口腔病变的风险仍可能增加。要了解 XLH 及其治疗对牙齿健康的影响,还需要进一步的研究。
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来源期刊
BDJ Open
BDJ Open Dentistry-Dentistry (all)
CiteScore
3.70
自引率
3.30%
发文量
34
审稿时长
30 weeks
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