Early exfoliation of permanent tooth in patient with hypophosphatasia. Issue 3 (December 2017)

• Record Type: Journal Article
• Authors: Okawa, Rena; Miura, Jiro; Kokomoto, Kazuma; Kubota, Takuo; Kitaoka, Taichi; Ozono, Keiichi; Nakano, Kazuhiko
• Is Part Of: Pediatric dental journal. Volume 27:Issue 3(2017)
• Page Start: 173
• Page End: 178
• Publication Date: 2017-12
• Subjects: Hypophosphatasia -- Early exfoliation -- Hypo-mineralization -- Disturbed cementum -- Permanent teeth; Pedodontics -- Periodicals; Pediatric Dentistry -- Abstracts; Pediatric Dentistry -- Periodicals; 617.645005
• DOI: 10.1016/j.pdj.2017.08.005
• Languages: English
• ISSNs: 0917-2394

Abstract

Abstract: Background: Hypophosphatasia (HPP) is a rare inherited skeletal disorder caused by mutations in the ALPL gene encoding tissue-nonspecific alkaline phosphatase, with early exfoliation of primary teeth due to disturbed formation of cementum often recognized as a major dental manifestation. However, reports regarding permanent teeth in HPP cases are scant. Case report: An 11-year-old boy diagnosed with childhood type HPP was referred to our hospital for exfoliation of the maxillary right central incisor. Micro-computed tomography findings of the affected tooth revealed external root resorption, enamel hypoplasia, thin dentin, and a wide pulp chamber, while disturbed cementum formation, enamel hypoplasia, dentin hypo-mineralization, and scant cementum around the enamel junction were observed by scanning electron microscopy. Conclusion: Permanent teeth may have a risk of early exfoliation as well as other structural abnormalities in HPP patients, thus longitudinal dental follow-up examinations of affected patients are required.