Pulmonary veno-occlusive disease and/or pulmonary capillary hemangiomatosis
Synonyms: Pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis
- Type
- Category
- Group
- Group of disorders
- Age of onset
- All ages
- Status
- active
A disorder that constitutes a rare subgroup of rare pulmonary hypertension characterized by obliterative fibrosis of the small pulmonary veins and venules and/or capillary infiltration of the pulmonary interstitium leading to increased pulmonary vascular resistance and right ventricular dysfunction.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Pulmonary veno-occlusive disease and/or pulmonary capillary hemangiomatosis | Pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis |
| fr | Maladie veino-occlusive pulmonaire et/ou hémangiomatose capillaire pulmonaire | |
| de | Pulmonale Venenverschlusskrankheit und/oder pulmonale kapilläre Hämangiomatose | |
| es | Enfermedad veno-oclusiva pulmonar y/o hemangiomatosis capilar pulmonar | |
| it | Malattia veno-occlusiva polmonare e/o emangiomatosi capillare polmonare | |
| nl | Pulmonale veno-occlusieve ziekte en/of pulmonale capillaire hemangiomatose | |
| pt | Doença veno-oclusiva pulmonar e / ou hemangiomatose capilar pulmonar | |
| pl | Żylno-okluzyjna choroba płuc i(lub) hemangiomatoza kapilarna płucna | = en |
| cs | Pulmonální venookluzní choroba a/nebo pulmonální kapilární hemangiomatóza | = en |
| uk | Легенева венооклюзивна хвороба та/або легеневий капілярний гемангіоматоз | |
| zh | 肺静脉闭塞性疾病和/或肺毛细血管血管瘤病 | |
| tr | Pulmoner ven-tıkayıcı hastalık ve/veya pulmoner kapiller hemanjiyomatoz |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| MONDO | 0018554 | E | validated | |
| MONDO | 18554 | E | validated | |
| UMLS | C3698354 | 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:156610Rare genetic respiratory disease
rare genetic diseases - ORPHA:71198Rare pulmonary hypertension
rare respiratory diseases
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | <1 / 1 000 000 | 0.015 / 100 000 | France | Value and class | Validated | 20456932[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
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/431353
- This record as JSON: /data/d/431353.json