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Dentin dysplasia

Disease definition

Dentin dysplasia (DD) is a rare disorder belonging to the group of hereditary dentin defects (see this term) and is characterized by abnormal dentin structure and root development resulting in abnormal tooth development. It encompasses two subtypes: DD type I and DD type II (see these terms).

ORPHA:1653

  • Synonym(s):
    • DD
  • Prevalence: Unknown
  • Inheritance: Autosomal dominant 
  • Age of onset: All ages
  • ICD-10: K00.5
  • OMIM: 125400  125420
  • UMLS: C0011430
  • MeSH: D003805
  • GARD: -
  • MedDRA: -

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