Figure 2

Figure 2 : 

Panoramic X rays.

2A) Child, aged 6 years, with odontohypophosphatasia, which resulted in the gradual loss of the mandibular deciduous incisors, with the first tooth lost at about 30 months. To be noted: marked alveolysis of the maxillary deciduous incisors, which were loose. There was a familial history of early tooth loss on the maternal side (mother and grandmother). The child’s dentition was deciduous. No other dental abnormality was observed. Genetic diagnostics confirmed the presence of an heterozygous mutation c.3o1T>G (p.Y1o1D ) of the ALPL gene, a severe mutation with a probable negative dominant effect.

2B) Child with a severe perinatal form of hypophosphatasia. Early tooth loss affected almost all of the deciduous teeth. The child’s dentition was mixed. The deciduous molars (54, 85 crowned, 75) persisted on the arch. The enamel was malformed. The permanent molars presented with taurodontism including elongation of the crown and of the the pulp chamber along the axis of the tooth, and within the apical branching of the roots.