Secondary sclerosing cholangitis
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Status
- active
A rare, biliary tract disease characterized by development of sclerosing cholangitis due to a known primary insult to the biliary tree, including infections, autoimmune disease, exposure to toxic agents, obstructive and ischemic injuries. Patients may be initially asymptomatic with only elevated alkaline phosphatase and gamma glutamyltransferase levels. Later presentation includes abdominal pain, jaundice, pruritus, fever and bacterial cholangitis from ascending infection.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Secondary sclerosing cholangitis | |
| fr | Cholangite sclérosante secondaire | |
| de | Sklerosierende Cholangitis, sekundäre | |
| es | Colangitis esclerosante secundaria | |
| it | Colangite sclerosante secondaria | |
| nl | Secundaire scleroserende cholangitis | |
| pt | Colangite esclerosante secundária | |
| pl | Wtórne twardniające zapalenie dróg żółciowych | |
| cs | Sekundární sklerotizující cholangitida | |
| uk | Вторинний склерозуючий холангіт | |
| zh | 继发型硬化性胆管炎 | |
| tr | Sekonder sklerozan kolanjit |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | K83.0 | NTBT | inclusion | validated |
| ICD-11 | DC13 | NTBT | index | validated |
| MONDO | 0018647 | E | validated | |
| UMLS | C0400978 | 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:447771Sclerosing cholangitis
rare hepatic diseases; rare transplant-related disorders
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
0 recordsNo prevalence record in this release.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/447774
- This record as JSON: /data/d/447774.json