ORPHA:98981
Essential iris atrophy
- Type
- Clinical subtype
- Group
- Subtype of disorder
- Status
- active
A clinical variant of iridocorneal endothelial (ICE) syndrome, characterized by progressive iris atrophy and holes present on the surface of the iris, corneal edema, corectopia, uveal ectropion and anterior synechiae. Secondary glaucoma is also a common complication of the disease.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Essential iris atrophy | |
| fr | Atrophie essentielle de l'iris | |
| de | Irisatrophie, essentielle | |
| es | Atrofia esencial de iris | |
| it | Atrofia essenziale dell'iride | |
| nl | Essentiële irisatrofie | |
| pt | Atrofia da íris, essencial | |
| pl | Zanik tęczówki | |
| cs | Esenciální atrofie duhovky | |
| uk | Есенціальна атрофія райдужної оболонки | |
| zh | 特发性虹膜萎缩症 | |
| tr | Esansiyel iris atrofisi |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | H21.2 | NTBT | inclusion | validated |
| ICD-11 | 9A90.2 | NTBT | index | validated |
| MONDO | 0020371 | E | validated | |
| UMLS | C0271111 | 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 (1):
- ORPHA:64734Iridocorneal endothelial syndrome
rare developmental anomalies during embryogenesis; rare genetic diseases; rare ophthalmic disorders
No children.
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Genes
0 directOrphanet records no gene directly on this code.
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Prevalence and incidence
0 recordsNo prevalence record in this release.
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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/98981
- This record as JSON: /data/d/98981.json