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Dentinogenesis imperfecta limited to primary teeth

MedGen UID:
892338
Concept ID:
C4023558
Anatomical Abnormality
Synonym: Dentinogenesis imperfecta of baby teeth
 
HPO: HP:0011060

Definition

Developmental dysplasia of dentin affecting only the primary dentition. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDentinogenesis imperfecta limited to primary teeth

Conditions with this feature

Denticles
MedGen UID:
315928
Concept ID:
C1527284
Disease or Syndrome
Dentin dysplasia type II (DTDP2) is a defect of dentin formation in which the clinical appearance of the secondary teeth is normal, but the primary teeth may appear opalescent, similar to teeth affected by dentinogenesis imperfecta. The roots of the teeth are of normal shape and morphologic character. The pulp chambers and root canals of the anterior teeth and the premolars are shaped like thistle tubes because of the radicular extension of the pulp chamber. Most teeth show accumulations of pulp stones in these unusually shaped pulp chambers (summary by Kalk et al., 1998). Also see dentin dysplasia type I (DTDP1; 125400).
Dentin dysplasia-sclerotic bones syndrome
MedGen UID:
377618
Concept ID:
C1852201
Disease or Syndrome
Dentin dysplasia-sclerotic bones syndrome is a rare, genetic odontologic disease characterized by the clinical, radiographic, and histologic features of dentine dysplasia and osteosclerosis of all long bones, with heavy cortical bone and narrowed or occluded marrow spaces. There have been no further descriptions in the literature since 1977.

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