Rare Diseases Symptoms Automatic Extraction

Dentinogenesis imperfecta in children with osteogenesis imperfecta: a clinical and ultrastructural study.

[dentinogenesis imperfecta]

The aim of this study was to assess the correlation between osteogenesis imperfecta (OI) and dentinogenesis imperfecta (DI) from both a clinical and histological point of view, particularly clarifying the structural and ultrastructural dentine changes.Sixteen children (6-12 years aged) with diagnosis of OI were examined for dental alterations referable to DI. For each patient, the OI type (I, III, or IV) was recorded. Extracted or normally exfoliated primary teeth were subjected to a histological examination (to both optical microscopy and confocal laser-scanning microscopy).A total of ten patients had abnormal discolourations referable to DI: four patients were affected by OI type I, three patients by OI type III, and three patients by OI type IV. The discolourations, yellow/brown or opalescent grey, could not be related to the different types of OI. Histological exam of primary teeth showed severe pathological change in the dentin, structured into four different layers. A collagen defect due to odontoblast dysfunction was theorized to be on the base of the histological changes.There is no correlation between the type of OI and the type of discolouration. The underlying dentinal defect seems to be related to an odontoblast dysfunction.

Diseases presenting "primary teeth" symptom

  • dentin dysplasia
  • dentinogenesis imperfecta
  • kabuki syndrome
  • oligodontia
  • papillon-lefèvre syndrome

You can validate or delete this automatically detected symptom