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Dentinogenesis imperfecta

Disease definition

Dentinogenesis imperfecta (DGI) is a hereditary dentin defect (see this term) characterized by abnormal dentin structure resulting in abnormal tooth development.

ORPHA:49042

  • Synonym(s):
    • DGI
    • DGI without OI
    • DI
    • Dentinogenesis imperfecta without osteogenesis imperfecta
    • Non-syndromic DGI
    • Non-syndromic dentinogenesis imperfecta
    • Opalescent teeth without OI
    • Opalescent teeth without osteogenesis imperfecta
  • Prevalence: 1-5 / 10 000
  • Inheritance: Autosomal dominant 
  • Age of onset: Childhood
  • ICD-10: K00.5
  • OMIM: -
  • UMLS: C0011436  C2973527
  • MeSH: D003811
  • GARD: 6258
  • MedDRA: 10054013

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