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

J Oral Rehabil

Department of Odontology, Section of Orthodontics, Faculty of Health and Medical Sciences, University of Copenhagen, Copenhagen, Denmark.

Published: January 2025

AI Article Synopsis

  • Hypophosphatasia (HPP) is a rare genetic disorder that impacts bone and dental health, and this study focuses on the dental characteristics of adults with adult-onset HPP (aHPP) compared to healthy individuals.
  • Researchers conducted a case-control study involving 20 aHPP patients and 31 healthy controls, using interviews, clinical examinations, and imaging techniques to assess their oro-dental health.
  • Findings revealed that aHPP patients experience more tooth fractures, higher rates of dental caries, and early loss of teeth, along with specific dental issues like tooth opacity and a larger gap between the enamel-cement junction and bone level, indicating poorer dental health overall.

Article 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.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11680502PMC
http://dx.doi.org/10.1111/joor.13878DOI Listing

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Article Synopsis
  • Hypophosphatasia (HPP) is a rare genetic disorder that impacts bone and dental health, and this study focuses on the dental characteristics of adults with adult-onset HPP (aHPP) compared to healthy individuals.
  • Researchers conducted a case-control study involving 20 aHPP patients and 31 healthy controls, using interviews, clinical examinations, and imaging techniques to assess their oro-dental health.
  • Findings revealed that aHPP patients experience more tooth fractures, higher rates of dental caries, and early loss of teeth, along with specific dental issues like tooth opacity and a larger gap between the enamel-cement junction and bone level, indicating poorer dental health overall.
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