Idiopathic peliosis hepatis
Synonyms: Idiopathic peliosis hepatitis
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult, Childhood
- Status
- active
A rare vascular liver disease characterized by widespread or focal cystic dilatation of sinusoidal blood-filled spaces of the liver without any known cause. Lesions can vary in diameter between few millimeters and several centimeters. The condition may remain asymptomatic or manifest with complications including rupture and intraperitoneal hemorrhage, hepatomegaly, portal hypertension, cholestasis, and liver failure.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Idiopathic peliosis hepatis | Idiopathic peliosis hepatitis |
| fr | Péliose hépatique idiopathique | |
| de | Peliosis hepatis, idiopathische | |
| es | Peliosis hepática idiopática | Hepatitis peliótica idiopática |
| it | Peliosi epatica idiopatica | |
| nl | Idiopathische peliosis hepatis | Idiopathische peliosis hepatitis |
| pt | Peliose hepática idiopática | |
| pl | Prosowate wylewy krwawe wątroby | = en |
| cs | Idiopatická pelióza jater | Idiopathic peliosis hepatitis · Idiopatická hepatická pelióza |
| uk | Ідіопатичний пеліоз печінки | Ідіопатичний пеліозний гепатит |
| zh | 特发性紫癜性肝炎 | = en |
| tr | İdiyopatik peliosis hepatis | İdiyopatik peliosis hepatiti |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | K76.4 | NTBT | attributed | validated |
| ICD-11 | DB98.1 | NTBT | attributed | validated |
| MONDO | 0018809 | E | validated | |
| UMLS | C5680101 | 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:101938Rare vascular liver disease
rare hepatic diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
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.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/480524
- This record as JSON: /data/d/480524.json