Idiopathic ductopenia
Synonyms: Idiopathic adult ductopenia · IAD
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Status
- active
A rare biliary tract disease characterized by loss of interlobular bile ducts resulting in chronic cholestasis, without any known cause. Loss of less than 50% of interlobular bile ducts is associated with a mild disease course, while loss of the majority of ducts results in a severe form, potentially leading to cirrhosis and liver failure. Patients typically present as young or middle-aged adults with episodic jaundice, pruritus, and elevated liver enzymes.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Idiopathic ductopenia | Idiopathic adult ductopenia · IAD |
| fr | Ductopénie idiopathique | Ductopénie idiopathique de l'adulte |
| de | Duktopenie, idiopathische | IAD · Idiopathische Duktopenie des Erwachsenen |
| es | Ductopenia idiopática | Ductopenia idiopática del adulto |
| it | Duttopenia idiopatica | |
| nl | Idiopathische ductopenie | IAD · Idiopathische ductopenie bij volwassenen |
| pt | Ductopenia idiopática | |
| pl | Duktopenia idiopatyczna | IAD · Idiopatyczna duktopenia dorosłych |
| cs | Idiopatická duktopenie | IAD · Idiopathic adult ductopenia · Idiopatická duktopenie v dospělosti |
| uk | Ідіопатична дуктопенія | IAD · Ідіопатична дуктопенія у дорослих |
| zh | 特发性胆管发育不良 | 特发性成人胆管发育不良 |
| tr | İdiyopatik duktopeni | IAD · İdiyopatik erişkin duktopeni |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | K83.8 | NTBT | attributed | validated |
| ICD-11 | DC1Y | NTBT | attributed | validated |
| MONDO | 0018807 | E | validated | |
| UMLS | C0022230 | 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:101941Rare biliary tract disease
rare hepatic diseases - ORPHA:506210Rare disorder potentially indicated for liver transplant
rare transplant-related 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 / 1 000 000 | — | Worldwide | Class only | Validated | 32337312[PMID]_ORPHANET |
| Cases/families | 100 cases | Worldwide | Case(s) | Validated | 32337312[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/480512
- This record as JSON: /data/d/480512.json