Structural deformities of deciduous teeth in patients with hypophosphatemic vitamin D-resistant rickets

Structural deformities of deciduous teeth from patients with hypophosphatemic vitamin D-resistant rickets (HVDRR) (1 male and 2 female patients) were examined by means of transmitted light microscopy, contact microradiography, and x-ray microanalysis. Freshly extracted teeth were fixed in formalin and subsequently hemisected longitudinally through the midline. One half was prepared for ground sections and the other half for decalcified sections. Neither gross nor microscopic abnormalities were present in enamel of patients with HVDRR. The concentration of calcium and phosphorus and the calcium/phosphorus ratio of the enamel of patients with HVDRR were nearly equal to those of normal teeth, although the degree of radiopacity was less in HVDRR. On the other hand, numerous microscopic abnormalities in the dentin of patients with HVDRR were found, such as interglobular dentin, wide predentin zones, and tubular defects. The concentration of phosphorus in the dentin of a patient with familial HVDRR was extremely low. Furthermore, formation of reparative dentin was observed at the pulp horn of teeth in patients with HVDRR that had been subjected to definite attrition at the corresponding dentin site.