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Dentinogenesis imperfecta type 2

Disease definition

Dentinogenesis imperfecta type 2 (DGI-2) is a rare, severe form of dentinogenesis imperfecta (DGI, see this term) and is characterized by weakness and discoloration of all teeth.


Classification level: Subtype of disorder
  • Synonym(s):
    • Capdepont teeth
    • DGI-2
    • DI-2
    • Dentinogenesis imperfecta, Shields type 2
  • Prevalence: 1-5 / 10 000
  • Inheritance: Autosomal dominant 
  • Age of onset: Childhood
  • ICD-10: K00.5
  • OMIM: 125490  605594
  • UMLS: C2973527
  • MeSH: -
  • GARD: 12796
  • MedDRA: -

Detailed information


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