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Archives de pédiatrie
Volume 24, n° 5S2
pages 580-584 (mai 2017)
Doi : 10.1016/S0929-693X(18)30020-4
Hypophosphatasia: oral cavity and dental disorders
Hypophosphatasie : atteintes buccale et dentaire

Figure 1

Figure 1 : 

Dental tissues.

The teeth consist of four tissues which are distinct but have strong functional or biological links. Dentin, a tissue like bone, accounts for the majority of the organ. The coronal dentin is covered by enamel and the radicular dentin by a very thin layer of cementum. The dental pulp retains its odontogenesis properties throughout its life. The teeth are connected to alveolar bone by the alveolodental (or periodontal) ligament.

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.

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