Congenital Abnormality
dentinogenesis imperfecta
Subclass of:
Tooth Abnormalities
Definitions related to dentinogenesis imperfecta:
  • A congenital tooth development disorder caused by mutations in the DSPP gene. The teeth are weak, discolored, and translucent.
    NCI
    U.S. National Cancer Institute, 2021
  • An autosomal dominant disorder of tooth development characterized by opalescent dentin resulting in discoloration of the teeth. The dentin develops poorly with low mineral content while the pulp canal is obliterated.
    NLM Medical Subject Headings
    U.S. National Library of Medicine, 2025
  • Dentinogenesis imperfecta (DGI) is a hereditary dentin defect characterized by abnormal dentin structure resulting in abnormal tooth development.
    OrphaNet
    INSERM, 2021
  • Developmental dysplasia of dentin.
    Human Phenotype Ontology (HPO)
    The Human Phenotype Ontology Project, 2025
  • Dentinogenesis imperfecta is a condition characterized by teeth that are translucent and discolored (most often blue-grey or yellow-brown in color). Individuals with this disorder tend to have teeth that are weaker than normal, which leads to wear, breakage, and loss of teeth. This damage can include teeth fractures or small holes...
    NIH Genetic and Rare Diseases
    National Center for Advancing Translational Sciences
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