2022 Volume 60 Issue 3 Pages 129-134
Hypophosphatasia (HPP) is a congenital bone disorder caused by mutations in the gene encoding tissue nonspecific alkaline phosphatase (TNSALP). It is typically associated with premature loss of deciduous teeth; thus, abnormal findings in a dental examination often lead to the diagnosis of HPP. Routine management of patients is critical as the loss of deciduous teeth can have a negative impact on nutritional intake and daily activities. We report the case of a patient with a suspected diagnosis of HPP.
The patient was 5 years and 3 months old at the time of the initial examination. His mother had been diagnosed with, and treated for, HPP. A clinical examination performed at a pediatric clinic showed that the patient had a low level of alkaline phosphatase (ALP); however, genetic testing had not been performed.
At the initial examination, the patient was in stage IIA of Hellman's stage of dental development with significant movement of the right mandibular deciduous central incisor. Dental X-ray performed at the initial examination revealed significant alveolar bone resorption while the root of the right mandibular deciduous central incisor was intact. Thus, we proceeded with plaque control.
At 1 month after the initial examination, the patient lost the right mandibular deciduous central incisor.Due to significant movement of the teeth adjacent to the tooth lost at 5 years and 4 months of age, we placed a pediatric denture in the position as a space maintainer. Since we observed plaque buildup in the maxillary and mandibular anterior teeth, we advised the mother of the patient to help him finish tooth brushing.
With continued management, we observed that the right mandibular central incisor started to grow by the time the patient had reached the age of 5 years and 10 months.
