loading...| Preferred Name |
Primary sclerosing cholangitis |
|
| Synonyms |
PSC |
|
| Definitions |
Primary sclerosing cholangitis (PSC) is a rare, slowly progressive liver disease characterized by inflammation and destruction of the intra- and/or extra-hepatic bile ducts that lead to cholestasis, liver fibrosis, liver cirrhosis and ultimately liver failure. |
|
| ID |
http://www.orpha.net/ORDO/Orphanet_171 |
|
| alternative_term |
PSC |
|
| definition |
Primary sclerosing cholangitis (PSC) is a rare, slowly progressive liver disease characterized by inflammation and destruction of the intra- and/or extra-hepatic bile ducts that lead to cholestasis, liver fibrosis, liver cirrhosis and ultimately liver failure. |
|
| definition_citation |
Orphanet |
|
| expertlink |
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=171 |
|
| has_age_of_onset |
Adolescent Adult Childhood Elderly |
|
| has_inheritance |
Multigenic/multifactorial |
|
| hasDbXref |
ICD-10:K83.0 UMLS:C0566602 OMIM:613806 MedDRA:10036732 ICD-11:DB96.2 OMIM:602114 |
|
| label |
Primary sclerosing cholangitis |
|
| notation |
ORPHA:171 |
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| part_of | ||
| prefixIRI |
ORDO:Orphanet_171 |
|
| prefLabel |
Primary sclerosing cholangitis |
|
| present_in |
United Kingdom AND has_annual_incidence_average_value : 0.66 AND has_annual_incidence_range : 1-9 / 1 000 000 United Kingdom AND has_point_prevalence_average_value : 8.28 AND has_point_prevalence_range : 1-9 / 100 000 Netherlands AND has_annual_incidence_average_value : 0.5 AND has_annual_incidence_range : 1-9 / 1 000 000 Spain AND has_annual_incidence_average_value : 0.07 AND has_annual_incidence_range : <1 / 1 000 000 Norway AND has_annual_incidence_average_value : 1.3 AND has_annual_incidence_range : 1-9 / 100 000 Canada AND has_annual_incidence_average_value : 0.9 AND has_annual_incidence_range : 1-9 / 1 000 000 Singapore AND has_point_prevalence_average_value : 1.3 AND has_point_prevalence_range : 1-9 / 100 000 United States AND has_point_prevalence_average_value : 8.81 AND has_point_prevalence_range : 1-9 / 100 000 Sweden AND has_annual_incidence_average_value : 1.22 AND has_annual_incidence_range : 1-9 / 100 000 Norway AND has_point_prevalence_average_value : 8.5 AND has_point_prevalence_range : 1-9 / 100 000 Europe AND has_annual_incidence_average_value : 0.77 AND has_annual_incidence_range : 1-9 / 1 000 000 United States AND has_annual_incidence_average_value : 0.77 AND has_annual_incidence_range : 1-9 / 1 000 000 Europe AND has_point_prevalence_average_value : 7.84 AND has_point_prevalence_range : 1-9 / 100 000 Worldwide AND has_annual_incidence_average_value : 0.65 AND has_annual_incidence_range : 1-9 / 1 000 000 Spain AND has_point_prevalence_average_value : 0.22 AND has_point_prevalence_range : 1-9 / 1 000 000 Worldwide AND has_point_prevalence_average_value : 8.1 AND has_point_prevalence_range : 1-9 / 100 000 Sweden AND has_point_prevalence_average_value : 16.2 AND has_point_prevalence_range : 1-5 / 10 000 Netherlands AND has_point_prevalence_average_value : 6.0 AND has_point_prevalence_range : 1-9 / 100 000 |
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| treeView | ||
| subClassOf |