ORPHA:694228
Congenital intrahepatic arterioportal fistula
Synonyms: IAPF
- Type
- Malformation syndrome
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Childhood, Infancy
- Status
- active
Orphadata ships no English definition for this code.
1
Names in Orphadata languages
9 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Congenital intrahepatic arterioportal fistula | IAPF |
| fr | Fistule artérioportale intrahépatique congénitale | = en |
| de | Kongenitale intrahepatische arterioportale Fistel | = en |
| es | Fístula arterioportal intrahepática congénita | FAP intrahepática |
| it | Fistola artero-portale intraepatica congenita | = en |
| nl | Congenitale intrahepatische arterioportale fistel | = en |
| pt | Fístula arterioporta intra-hepática congénita | = en |
| pl | Wrodzona wewnątrzwątrobowa przetoka tętniczo-wrotna | = en |
| cs | Congenital intrahepatic arterioportal fistula | = en |
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | Q26.6 | E | specific | validated |
| ICD-11 | LA90.30 | E | specific | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
3
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (2):
- ORPHA:101938Rare vascular liver disease
rare hepatic diseases - ORPHA:693855Visceral fast-flow vascular malformation
rare developmental anomalies during embryogenesis; rare circulatory system diseases
No children.
4
Genes
0 directOrphanet records no gene directly on this code.
5
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Cases/families | 60 cases | Worldwide | Case(s) | Validated | ORPHANET | |
| Point prevalence | <1 / 1 000 000 | — | 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/694228
- This record as JSON: /data/d/694228.json