Dental abnormalities in patients with familial hypophosphatemic vitamin D-resistant rickets: Prevention by early treatment with 1-hydroxyvitamin D

Objective To evaluate the dental effects of 1-hydroxylated vitamin D3 treatment in patients with familial hypophosphatemic vitamin D–resistant rickets. Study design Forty-eight children and adult patients were included in the study; 16 had received no treatment or phosphate supplements with vitamin D/25-(OH) D3 before puberty. The 32 younger ones had received phosphate supplements with 1α-(OH)D3 from infancy. All patients were clinically examined, and panoramic and periapical radiographs were made. Evaluations of decayed, missing, or filled teeth and decayed or filled teeth indexes and of pulp ratios allowed comparison with healthy age-matched control patients. Results Poor dental health and characteristic dental anomalies were found in the 16 older patients. In contrast, the 32 younger patients had a normal dental status as regards reference ranges in healthy age-matched populations, although they still showed prominent pulp horns on deciduous teeth and increased pulp area/tooth area ratios. Conclusions This investigation shows the beneficial effects of 1α-(OH)D3 treatment on the dental status of vitamin D–resistant rickets patients and emphasizes the necessity of early treatment. Remaining defects may result from early exposure of odontoblasts and surrounding osteoblasts to hypophosphatemia, before the commencement of treatment, and/or from intrinsic cell disturbances linked to the genetic alteration(s). (J Pediatr 2003;142:324-31)