Oro-Dental Characteristics in Patients With Adult-Onset Hypophosphatasia Compared to a Healthy Control Group-A Case-Control Study

J Oral Rehabil. 2025 Jan;52(1):64-74. doi: 10.1111/joor.13878. Epub 2024 Oct 11.

Abstract

Background: Hypophosphatasia (HPP) is a rare inherited disease that affects multiple organ systems including bone and teeth. Limited knowledge exists on dental and oral health in patients with adult-onset HPP (aHPP).

Objective: The aim of this study was to investigate oro-dental characteristics in patients with aHPP compared to healthy controls.

Methods: This case-control study included 20 patients with aHPP compared to 31 healthy controls. Oro-dental manifestations were examined by standardised interviews, clinical examinations as well as radiological registrations on panoramic radiograph (OP) and cone-beam computed tomography (CBCT) scans.

Results: The subjective experience of tooth fractures (p = 0.010), caries in permanent teeth (p = 0.032) and early loss of permanent teeth (p = 0.002) was significantly higher in patients with aHPP compared to the controls. In the aHPP group, the presence of specific teeth (p ≤ 0.045) and attrition of 11 were significantly lower (p = 0.012) compared to the controls. Opacity of a few teeth (p ≤ 0.049), presence of denticles (p = 0.024), the distance between the enamel-cement junction (CEJ) and the marginal bone level at specific sites (p ≤ 0.021) and crown height of 11 (p = 0.017) were significantly higher in patients with aHPP than in healthy controls.

Conclusion: The results indicate that patients with aHPP have a subjective experience of having poorer dental health. Loss of permanent teeth, less attrition, tooth opacities, denticles and larger distance between CEJ and marginal bone level are possible oro-dental findings in patients with aHPP.

Keywords: CBCT; adult‐onset; hypophosphatasia; oro‐dental characteristics.

MeSH terms

  • Adult
  • Case-Control Studies
  • Cone-Beam Computed Tomography*
  • Dental Caries / complications
  • Female
  • Humans
  • Hypophosphatasia* / complications
  • Hypophosphatasia* / physiopathology
  • Male
  • Middle Aged
  • Oral Health*
  • Radiography, Panoramic*
  • Tooth Fractures / diagnostic imaging
  • Tooth Loss

Supplementary concepts

  • Hypophosphatasia, Adult