Dental manifestation and management of hypophosphatasia

Okawa, Rena; Nakano, Kazuhiko

doi:10.1016/j.jdsr.2022.06.002

Cited by 9 publications

(3 citation statements)

References 107 publications

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“…Insufficient bone development, manifested by smaller and/or inconsistent dental arches, is most likely the cause of crowding of the teeth and/or mesial occlusion (Class III) [47]. In addition, an open bite or impacted or ectopic eruption of the maxillary canines is frequently observed in patients with delayed growth of the maxilla compared to the mandible [48]. In some cases in this study, the timing of tooth eruption was also somewhat unusual.…”

Section: Discussionmentioning

confidence: 61%

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

Arhar,

Pavlič,

Hočevar

2024

BDJ Open

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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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Section: Discussionmentioning

confidence: 61%

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

Arhar,

Pavlič,

Hočevar

2024

BDJ Open

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“…Studies have shown that treatment with asfotase alfa injection therapy improves dental mineralization, resulting in the stabilization of periodontal tissues and ultimately resulting in better growth of tooth roots. [4][5][6][7] Treatment with asfotase alfa injection is FDA approved for patients with perinatal/infantile and juvenile-onset hypophosphatasia and proven to improve respiratory function, motor function, and bone mineralization. 3 This case highlights the importance of early diagnosis of hypophosphatasia in adults and appropriate treatment to prevent further complications.…”

Section: What Is the Diagnosis?mentioning

confidence: 99%

“…In these patients with early exfoliation of primary teeth, dentures are generally recommended so as to ensure that important oral functions are acquired. Studies have shown that treatment with asfotase alfa injection therapy improves dental mineralization, resulting in the stabilization of periodontal tissues and ultimately resulting in better growth of tooth roots 4–7 . Treatment with asfotase alfa injection is FDA approved for patients with perinatal/infantile and juvenile‐onset hypophosphatasia and proven to improve respiratory function, motor function, and bone mineralization 3 .…”

Section: What Is the Diagnosis?mentioning

confidence: 99%

Dental loss, stress fractures, and musculoskeletal pain in a 48‐year‐old woman

Hoang

Hatfield

Nguyen

et al. 2023

Clinical Case Reports

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Role of PLP-Level as a predictive marker for oral health status in adult hypophosphatasia

Dudde,

Fildebrandt,

Smeets

et al. 2024

Clin Oral Invest

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Aim The aim of this study was to investigate the role of pyridoxal-5-phosphate (PLP) level on the oral health status as a predictive marker in patients with hypophosphatasia (HPP). Materials and methods Throughout a systematic retrospective assessment both bone metabolism and oral health status were analyzed. The oral health status was assessed by the decayed/missing/filled teeth index (DMFT), clinical attachment level (CAL), probing pocket depth (PPD), and the periodontal screening index (PSI). Results A total of 48 HPP patients (81.3% female) with a mean age of 42.21 years was included in this retrospective study. The study population was divided into two groups using the mean PLP level (87 µg/l) as a cut-off. Patients with a PLP level ≥ 87 µg/l (n = 14) showed a significantly poorer oral health status regarding DMFT index, CAL, PPD and PSI compared to patients with a PLP level < 87 µg/l (n = 34). No significant group differences for tooth loss were found. Conclusion The results of the present study indicate that the PLP level is a suitable diagnostic predictor for the oral health status in HPP patients. HPP patients with PLP levels ≥ 70 µg/l should be included into a regular dental preventive program. Clinical Relevance The oral health status in HPP and its correlation with laboratory parameters (i.e. PLP) has been understudied. For clinical practice, the findings of the present study clearly demonstrated that high PLP levels correlate with a worse oral health status in HPP patients. Therefore, these patients should receive an intensive dental treatment and/or inclusion in a strict maintenance program in a specialized dental practice/university hospital with a PLP level ≥ 70 µg/l.

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Dental manifestation and management of hypophosphatasia

Cited by 9 publications

References 107 publications

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

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

Dental loss, stress fractures, and musculoskeletal pain in a 48‐year‐old woman

Role of PLP-Level as a predictive marker for oral health status in adult hypophosphatasia

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