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Trichodental syndrome

Disease definition

A rare ectodermal dysplasia syndrome characterized by the association of sparse, fine, dry, slow growing hair with variable dental abnormalities including oligodontia, peg-shaped incisors, and shell teeth. Mild intellectual disability, microcephaly, and dysmorphic facial features have also been reported.

ORPHA:3351

Classification level: Disorder
  • Synonym(s):
    • Kersey syndrome
  • Prevalence: <1 / 1 000 000
  • Inheritance: Autosomal dominant 
  • Age of onset: Childhood
  • ICD-10: -
  • OMIM: 601453
  • UMLS: C0406724
  • MeSH: C536551
  • GARD: 265
  • MedDRA: -
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