[Dentofacial manifestations in children with vitamin D-dependent Rickets type II].

Calcification of the teeth, size of the teeth and dental arches, facial growth and calcification of the carpal bones were studied in three children with clinically different severities of vitamin D-dependent rickets (DDR), type II, with alopecia, which is 1,25-dihydroxyvitamin D-receptor-defect rickets and is particularly resistant to treatment with calciferol analogues. They were treated in a pediatric clinic with large doses of 1 alpha-hydroxyvitamin D3(1 alpha-(OH)D3) and 2 g/day of calcium lactate. The results were as follows: 1. Hypoplasia of enamel of the deciduous teeth was not found. 2. In the deciduous teeth, large pulp chambers and thin dentin were seen in radiographs before treatment. In patients 1 and 2, these abnormalities were reversed by treatment. In patient 3, who had the severest manifestations, large pulp chambers and thin dentin decreased but still remained. 3. Growth of permanent teeth was retarded before treatment and during resistance to treatment. After effective medication, it caught up and was corrected. 4. Problems concerning maxillary and mandibular growth were not found. However Nasions of three patients were more front and lower and Orbitals were lower than standard. 5. In patients 1 and 2, the calcification of carpal bones was accelerated and in patient 3 retarded. 6. Mesiodistal dimensions of erupted deciduous and permanent teeth were within the standard range, except for patient 3, who had smaller lower deciduous teeth. 7. A ground section of the extracted upper right first deciduous molar from patient 3 showed abundant inter-globular dentin and lack of pre-dentinal layer. From the above findings, it was felt that in all probability dentinogenesis was disturbed by the DDR type II. Abnormally large pulp chambers and thin dentin could be corrected by effective medication. In patients with vitamin D-dependent rickets type II, oral hygiene for caries prevention is the most important procedure, because the pulp will be infected immediately after initiation of dental caries. After effective medication, permanent teeth and jaw bones will probably grow normally.