Hereditary dentin defect
- Type
- Category
- Group
- Group of disorders
- Status
- active
A group of rare disorders comprises dentinogenesis imperfecta and dentin dysplasia characterized by abnormal dentin structure affecting either the primary or both the primary and secondary dentitions. Clinically, the teeth are discolored and exhibit structural defects, such as bulbous crowns and small pulp chambers on radiographs. The underlying mineralization defect often results in shearing of the overlying enamel, leaving weakened dentin exposed and prone to wear.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Hereditary dentin defect | |
| fr | Anomalie héréditaire de la dentine | |
| de | Dentindefekt, hereditärer | |
| es | Defecto hereditario de la dentina | |
| it | Difetto ereditario della dentina | |
| nl | Erfelijk dentinedefect | |
| pt | Deficiência hereditária da dentina | |
| pl | Wrodzony defekt zębiny | |
| cs | Vrozené vady dentinu | |
| uk | Спадковий дефект дентину | |
| zh | 遗传性牙本质缺损 | |
| tr | Kalıtsal dentin defekti |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| MONDO | 15668 | E | validated | |
| UMLS | C5680465 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (2):
- ORPHA:420755Rare genetic odontal or periodontal disorder
rare genetic diseases - ORPHA:164001Rare odontal or periodontal disorder
rare odontological diseases
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
0 recordsNo prevalence record in this release.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/167759
- This record as JSON: /data/d/167759.json