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Dentin dysplasia-sclerotic bones syndrome

MedGen UID:
377618
Concept ID:
C1852201
Disease or Syndrome
Synonyms: Dentin dysplasia sclerotic bones; Sclerotic bones with dentin dysplasia
 
Monarch Initiative: MONDO:0007438
OMIM®: 125440
Orphanet: ORPHA99792

Definition

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. [from ORDO]

Clinical features

From HPO
Dentinogenesis imperfecta limited to primary teeth
MedGen UID:
892338
Concept ID:
C4023558
Anatomical Abnormality
Developmental dysplasia of dentin affecting only the primary dentition.
Cortical sclerosis
MedGen UID:
870710
Concept ID:
C4025164
Pathologic Function
Sclerosis (abnormal hardening) of cortical bone, characterized by increased radiodensity.
Abnormality of the dentition
MedGen UID:
78084
Concept ID:
C0262444
Finding
Any abnormality of the teeth.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDentin dysplasia-sclerotic bones syndrome
Follow this link to review classifications for Dentin dysplasia-sclerotic bones syndrome in Orphanet.

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