Neurotrophic keratopathy
Synonyms: Neurotrophic keratitis
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult, Childhood
- Status
- active
Neurotrophic keratopathy is a rare degenerative disease of the cornea characterized by reduction or loss of corneal sensitivity that can be asymptomatic or present with red-eye and, during the early stages of the disease, a minor decrease in visual acuity. It eventually leads to loss of vision.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Neurotrophic keratopathy | Neurotrophic keratitis |
| fr | Kératopathie neurotrophique | Kératite neurotrophique |
| de | Keratopathie, neurotrophe | Neurotrophe Keratitis |
| es | Queratopatía neurotrófica | Queratitis neurotrófica |
| it | Cheratopatia neurotrofica | |
| nl | Neurotrofe keratopathie | Neurotrofe keratitis |
| pt | Queratopatia neurotrófica | |
| pl | Keratopatia neurotropowa | Neurotropowe zapalenie rogówki |
| cs | Neurotrofická keratopatie | Neurotrofická keratitida |
| uk | Нейротрофічна кератопатія | Нейротрофічний кератит |
| zh | 神经营养性角膜病变 | 神经营养性角膜炎 |
| tr | Nörotrofik keratopati | Nörotrofik keratit |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | H16.2 | NTBT | attributed | validated |
| ICD-11 | 1F00.10 | NTBT | index | validated |
| MONDO | 0015290 | E | validated | |
| UMLS | C0339296 | E | validated | |
| MedDRA | 10069732 | 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:519278Infective keratitis
rare ophthalmic disorders
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | 1-5 / 10 000 | 4.2 / 100 000 | Europe | Value and class | Validated | European Medicines Agency 2015[OTHER] |
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | ORPHANET_24672223[PMID] |
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
15 annotations · validated 2019-06-28| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0010824 | Abnormal fifth cranial nerve morphology | |
| HP:0012155 | Decreased corneal sensation | |
| Frequent (79-30%) | ||
| HP:0007924 | Slow decrease in visual acuity | |
| Occasional (29-5%) | ||
| HP:0012122 | Anterior uveitis | |
| HP:0000483 | Astigmatism | |
| HP:0000622 | Blurred vision | |
| HP:0000559 | Corneal scarring | |
| HP:0012040 | Corneal stromal edema | |
| HP:0012804 | Corneal ulceration | |
| HP:0000819 | Diabetes mellitus | |
| HP:0000632 | Lacrimation abnormality | |
| HP:0000495 | Recurrent corneal erosions | |
| Very rare (<4-1%) | ||
| HP:0012533 | Allodynia | |
| HP:0100583 | Corneal perforation | |
| HP:0100963 | Hyperesthesia | |
Run the differential on the 2 obligate / very frequent terms
Annotation source: 28613758[PMID]_14631406[PMID]_29988739[PMID]_24672223[PMID]_29698813[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/137596
- This record as JSON: /data/d/137596.json