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PubMed · 3270649

[Dentinal dysplasia type I].

Abstract

Dentinal dysplasia is one of the rarest hereditary disturbances of dentin formation characterized by the teeth present with short blunt roots, considerably reduced or obliterated pulp spaces, periapical abscess or cysts without an obvious causative factor, and spontaneous exfoliated. These features were observed on a patient referred for prosthodontic assessment, and an investigation of other members of the family revealed that the father and two siblings were also affected. The report described the clinical, radiographic, histologic and scanning electron microscopic features and discussed the features which separated from dentinogenesis imperfecta.

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BibTeXRIS

D Tongnoi, T Triratana, S Arunakul. [Dentinal dysplasia type I].. https://pubmed.ncbi.nlm.nih.gov/3270649/

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Heritable dentin defects: nosology, pathology, and treatment.

Heritable dentin defects have been divided into 2 main categories: dentinogenesis imperfecta (DI) and dentin dysplasia (DD). Recent studies have shown that they share many features in common. Of the connective tissue diseases, only osteogenesis imperfecta (OI) has been linked to these disorders. So far, no definitive relation between the type of OI and the dental involvement can be established. Familial occurrence of DI with OI cannot be comprehensively explained by mutations in type I collagen genes. No information about the gene defects in DD is available. At the ultrastructural level, the organization of the normally cross-striated collagen fibers in the dentin matrix varies markedly in patients affected by DI.

Dentin Dysplasia↗