title: Hypophosphatasia affecting the permanent dentition.
abstract: Reports on dental abnormalities in connection with hypophosphatasia almost exclusively describe changes in primary teeth. A 23-year-old man with hypophosphatasia, first diagnosed at the age of 8 months, is described; histologically and radiographically verified signs of the condition were present in the permanent dentition. The findings included a reduced level of the marginal alveolar bone supporting the upper central incisors, which had to be extracted. The molars displayed large coronal pulp chambers. Histologically, the upper incisors demonstrated abnormal root cementum, with areas of dentin resorption, as well as disturbances of the mineralization of the coronal dentin. The patient also had signs of abnormal root resorption of molars. The potential involvement of permanent teeth puts children with hypophosphatasia at risk of developing oral complications during adolescent and adult life.
authors.affiliation: Department of Periodontology, Public Dental Health Service, Lulea, Sweden.
authors.forename: A
authors.initials: A
authors.lastname: Olsson
authors.forename: L
authors.initials: L
authors.lastname: Matsson
authors.forename: H K
authors.initials: HK
authors.lastname: Blomquist
authors.forename: A
authors.initials: A
authors.lastname: Larsson
authors.forename: B
authors.initials: B
authors.lastname: Sjödin
mesh_terms: D000328:Adult; D016301:Alveolar Bone Loss / Q000209:etiology; D003739:Dental Cementum / Q000002:abnormalities*; D003786:Dental Pulp Cavity / Q000002:abnormalities; D019229:Dentition, Permanent; D006801:Humans; D007014:Hypophosphatasia / Q000150:complications* / Q000235:genetics; D008297:Male; D008963:Molar / Q000002:abnormalities; D010375:Pedigree; D014071:Tooth Abnormalities / Q000209:etiology*; D019228:Tooth Crown / Q000002:abnormalities