ORPHA:98627
Posterior corneal dystrophy
- Type
- Category
- Group
- Group of disorders
- Inheritance
- Autosomal dominant, Autosomal recessive, X-linked recessive
- Age of onset
- All ages
- Status
- active
Posterior corneal dystrophies refers to a group of rare genetically determined corneal dystrophies (CDs) characterized by lesions affecting the corneal endothelium and Descemet membrane, and variable effects on vision depending on the type of dystrophy.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Posterior corneal dystrophy | |
| fr | Dystrophie cornéenne postérieure | |
| de | Hornhautdystrophie, posteriore | |
| es | Distrofia corneal posterior | |
| it | Distrofia corneale posteriore | |
| nl | Posterieure corneadystrofie | |
| pt | Distrofia da córnea posterior | |
| pl | Dystrofia tylna rogówki | |
| cs | Zadní korneální dystrofie | |
| uk | Задня дистрофія рогівки | |
| zh | 后角膜营养不良 | |
| tr | Posterior kornea distrofisi |
Definition in:
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Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| MONDO | 0020214 | E | validated | |
| UMLS | C2063478 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
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Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (2):
- ORPHA:34533Corneal dystrophy
rare ophthalmic disorders - ORPHA:522560Genetic corneal dystrophy
rare genetic diseases
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Genes
0 directOrphanet records no gene directly on this code.
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Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | 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.
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HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/98627
- This record as JSON: /data/d/98627.json