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Dentinogenesis imperfecta type 3

Disease definition

Dentinogenesis imperfecta type 3 (DGI-3) is a rare, severe form of dentinogenesis imperfecta (DGI, see this term) characterized by opalescent primary and permanent teeth, marked attrition, large pulp chambers, multiple pulp exposure and shell teeth radiographically (i.e. teeth which appear hollow due to dentin hypotrophy).

ORPHA:166265

  • Synonym(s):
    • Dentinogenesis imperfecta, Shields type 3
  • Prevalence: Unknown
  • Inheritance: Autosomal dominant 
  • Age of onset: Childhood
  • ICD-10: K00.5
  • OMIM: 125500
  • UMLS: C0399378
  • MeSH: -
  • GARD: -
  • MedDRA: -

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