Oro-Dental Characteristics in Patients With Adult-Onset Hypophosphatasia Compared to a Healthy Control Group-A Case-Control Study.
PMID 39394635 · Journal of oral rehabilitation · 2025 · DOI 10.1111/joor.13878
Authors: Freja Fribert Jørgensen; Xenia Hermann; Nicola Hepp; Liselotte Sonnesen
Abstract
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). The aim of this study was to investigate oro-dental characteristics in patients with aHPP compared to healthy controls. 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. 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. 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.
Humans; Case-Control Studies; Hypophosphatasia; Male; Female; Adult; Cone-Beam Computed Tomography; Oral Health; Radiography, Panoramic; Middle Aged; Tooth Fractures; Dental Caries; Tooth Loss
Cite
Freja Fribert Jørgensen; Xenia Hermann; Nicola Hepp; Liselotte Sonnesen. Oro-Dental Characteristics in Patients With Adult-Onset Hypophosphatasia Compared to a Healthy Control Group-A Case-Control Study. Journal of oral rehabilitation (2025). PMID 39394635. DOI 10.1111/joor.13878.