Dentinogenesis imperfecta type 3

General Information (adopted from Orphanet):

Synonyms, Signs: DGI-III
BRANDYWINE TYPE DENTINOGENESIS IMPERFECTA
Dentinogenesis imperfecta, Shields type 3
Number of Symptoms 14
OrphanetNr: 166265
OMIM Id: 125500
ICD-10: K00.5
UMLs:
MeSH:
MedDRA:
Snomed:

Prevalence, inheritance and age of onset:

Prevalence: No data available.
Inheritance: Autosomal dominant
[Orphanet]
Age of onset: Childhood
[Orphanet]

Disease classification (adopted from Orphanet):

Parent Diseases: Dentinogenesis imperfecta
 -Rare genetic disease
 -Rare odontologic disease

Symptom Information: Sort by abundance 

1
 (HPO:0000700) Periapical radiolucency 2 / 7739
2
 (HPO:0000703) Dentinogenesis imperfecta 18 / 7739
3
 (HPO:0000694) Shell teeth 2 / 7739
4
 (OMIM) Pulp exposure 2 / 7739
5
 (OMIM) Normal-to-enlarged pulp chamber (primary) 1 / 7739
6
 (OMIM) Incisal notching 1 / 7739
7
 (OMIM) Anterior openbite 1 / 7739
8
 (OMIM) Obliterated pulp chamber (secondary) 1 / 7739
9
 (OMIM) Enamel pitting 6 / 7739
10
 (OMIM) Primary and secondary teeth affected 2 / 7739
11
 (HPO:0000006) Autosomal dominant inheritance 2518 / 7739
12
 (OMIM) Bulbous tooth crowns 1 / 7739
13
 (OMIM) Amber-opalescent colored teeth (primary and secondary) 1 / 7739
14
 (OMIM) Marked attrition (primary and secondary) 1 / 7739

Associated genes:

ClinVar (via SNiPA)

Gene symbol Variation Clinical significance Reference

Additional Information: