DENTINOGENESIS IMPERFECTA 1
General Information (adopted from Orphanet):
Synonyms, Signs: |
OPALESCENT TEETH WITHOUT OSTEOGENESIS IMPERFECTA OPALESCENT DENTIN CAPDEPONT TEETH DENTINOGENESIS IMPERFECTA, SHIELDS TYPE II DENTINOGENESIS IMPERFECTA WITHOUT OSTEOGENESIS IMPERFECTA DGI-II DGI1 |
Number of Symptoms | 8 |
OrphanetNr: | |
OMIM Id: |
125490
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ICD-10: |
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UMLs: |
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MeSH: |
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MedDRA: |
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Snomed: |
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Prevalence, inheritance and age of onset:
Prevalence: | No data available. |
Inheritance: |
Autosomal dominant inheritance [Omim] |
Age of onset: |
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Disease classification (adopted from Orphanet):
Parent Diseases: | No data available. |
Symptom Information:
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(HPO:0000703) | Dentinogenesis imperfecta | 18 / 7739 | ||||
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(OMIM) | Bulbous shaped crown | 1 / 7739 | ||||
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(OMIM) | Absent pulp chambers | 2 / 7739 | ||||
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(OMIM) | Brown-blue or opalescent brown teeth | 1 / 7739 | ||||
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(OMIM) | Narrow roots | 1 / 7739 | ||||
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(OMIM) | Root canals are small or obliterated | 1 / 7739 | ||||
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(OMIM) | Primary and secondary teeth affected | 2 / 7739 | ||||
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(OMIM) | Severe attrition | 1 / 7739 |
Associated genes:
ClinVar (via SNiPA)
Gene symbol | Variation | Clinical significance | Reference |
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