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.

Hypophosphatasia: an overview of the disease and its treatment

“…It can be inherited as an autosomal recessive or autosomal dominant disease [51,78,79]. The clinical presentation is with dental alterations (premature exfoliation of primary teeth and/or severe dental caries) without evidence of bone disease such as rickets or osteomalacia.…”

“…The enamel seems not to be directly affected in HPP patients, although enamel hypoplasia, predisposing to dental caries, has been reported. Even if the primary teeth are most characteristically affected, HPP may also cause alterations in the permanent teeth, requiring counseling and early interventions [49][50][51][52][53].…”

Systemic disorders and their influence on the development of dental hard tissues: A literature review

“…It has also been reported that dental effects of hypophosphatasia first diagnosed in primary teeth can also be seen in the permanent dentition. 58 Both histological and radiological changes, with a reduced level of marginal alveolar bone supporting the upper central incisors, large coronal pulp chambers in the molars, abnormal root cementum and dentin resorption and mineralisation disturbances have been observed in a young man with hypophosphatasia. 58 It is clear that moderate forms of hypophosphatasia are highly variable in their clinical expression, owing in part to allelic heterogeneity but also to other factors that remain undetermined at this time.…”

“…In addition, odontohypophosphatasia is a disease in which children and adults have only dental, not skeletal, problems, usually involving premature loss of teeth and/or wide pulp chambers that predisposes them to cavities and dental caries. 52,53,58,59 The clinical forms of this disease tend to have different modes of history and presentation. However, both autosomal recessive and autosomal dominant patterns of inheritance have been demonstrated for the childhood, adult and odontohypophosphatasia forms.…”

Prosthetic Rehabilitation of Hypophosphatasia with Precision Attachment Retained Unconventional Partial Denture: A Case Report

“…Hypophosphatasia is a rare metabolic bone disease that is characterized by deficient activity of tissue-nonspecific alkaline phosphatase (TNSALP) [8]. This deficient activity of TNSALP has been attributed to mutations in TNSALP gene which causes significant changes in the structure and functions of TNSALP [9].…”