Segmental odontomaxillary dysplasia
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Childhood
- Status
- active
A rare oral disease characterized by unilateral enlargement of the right or left maxillary alveolar bone and gingiva in the region distal to the canines towards the maxillary tuberosity. In the enlarged region, dental abnormalities such as missing teeth, abnormal spacing and delayed eruption occur.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Segmental odontomaxillary dysplasia | |
| fr | Dysplasie odonto-maxillaire segmentaire | |
| de | Dysplasie, odonto-maxilläre segmentale | |
| es | Displasia odontomaxilar segmentaria | |
| it | Displasia odontomascellare segmentale | |
| nl | Segmentale odontomaxillaire dysplasie | SOD |
| pt | Displasia odontomaxilar segmentar | DOS |
| pl | Segmentalna dysplazja zębowo-szczękowa | SOD |
| cs | Segmentální odontomaxilární dysplázie | SOD |
| uk | Сегментарна одонтомаксилярна дисплазія | |
| zh | 节段性牙颌骨异型增生 | |
| tr | Segmental odontomaksiller displazi |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | K00.4 | NTBT | attributed | validated |
| ICD-11 | DA07.3 | NTBT | index | validated |
| MONDO | 0019029 | E | validated | |
| UMLS | C3698531 | 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 (1):
- ORPHA:164001Rare odontal or periodontal disorder
rare odontological diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Cases/families | 32 cases | Worldwide | Case(s) | Validated | ORPHANET | |
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | Validated | ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/67039
- This record as JSON: /data/d/67039.json