Preferred Name |
Primary biliary cholangitis |
|
Synonyms |
PBC |
|
Definitions |
A rare autoimmune cholestatic liver disease characterized by autoimmune mediated damage of small intrahepatic bile ducts leading to cholestasis, fibrosis, and potential cirrhosis. |
|
ID |
http://www.orpha.net/ORDO/Orphanet_186 |
|
alternative_term |
PBC Primary biliary cirrhosis Hanot syndrome |
|
definition |
A rare autoimmune cholestatic liver disease characterized by autoimmune mediated damage of small intrahepatic bile ducts leading to cholestasis, fibrosis, and potential cirrhosis. |
|
definition_citation |
Orphanet |
|
expertlink |
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=186 |
|
has_age_of_onset |
Adolescent Adult Elderly |
|
has_inheritance |
Multigenic/multifactorial Unknown |
|
hasDbXref |
OMIM:614220 OMIM:614221 UMLS:C0008312 ICD-10:K74.3 OMIM:613008 OMIM:613007 OMIM:109720 ICD-11:DB96.1 MedDRA:10080429 MeSH:D008105 |
|
label |
Primary biliary cholangitis |
|
notation |
ORPHA:186 |
|
part_of | ||
prefixIRI |
ORDO:Orphanet_186 |
|
prefLabel |
Primary biliary cholangitis |
|
present_in |
Iceland AND has_point_prevalence_average_value : 38.3 AND has_point_prevalence_range : 1-5 / 10 000 Spain AND has_annual_incidence_average_value : 1.7 AND has_annual_incidence_range : 1-9 / 100 000 Worldwide AND has_annual_incidence_average_value : 3.0 AND has_annual_incidence_range : 1-9 / 100 000 Denmark AND has_annual_incidence_average_value : 1.7 AND has_annual_incidence_range : 1-9 / 100 000 Spain AND has_point_prevalence_average_value : 19.5 AND has_point_prevalence_range : 1-5 / 10 000 Netherlands AND has_point_prevalence_average_value : 13.2 AND has_point_prevalence_range : 1-5 / 10 000 Brunei Darussalam AND has_annual_incidence_average_value : 1.0 AND has_annual_incidence_range : 1-9 / 100 000 United States AND has_point_prevalence_average_value : 40.2 AND has_point_prevalence_range : 1-5 / 10 000 United Kingdom AND has_point_prevalence_average_value : 25.0 AND has_point_prevalence_range : 1-5 / 10 000 Netherlands AND has_annual_incidence_average_value : 1.1 AND has_annual_incidence_range : 1-9 / 100 000 United States AND has_annual_incidence_average_value : 4.5 AND has_annual_incidence_range : 1-9 / 100 000 United Kingdom AND has_annual_incidence_average_value : 2.3 AND has_annual_incidence_range : 1-9 / 100 000 Europe AND has_annual_incidence_average_value : 2.57 AND has_annual_incidence_range : 1-9 / 100 000 Finland AND has_point_prevalence_average_value : 18.0 AND has_point_prevalence_range : 1-5 / 10 000 Finland AND has_annual_incidence_average_value : 1.7 AND has_annual_incidence_range : 1-9 / 100 000 Canada AND has_point_prevalence_average_value : 22.7 AND has_point_prevalence_range : 1-5 / 10 000 Canada AND has_annual_incidence_average_value : 3.0 AND has_annual_incidence_range : 1-9 / 100 000 China AND has_point_prevalence_average_value : 4.9 AND has_point_prevalence_range : 1-9 / 100 000 Iceland AND has_annual_incidence_average_value : 2.5 AND has_annual_incidence_range : 1-9 / 100 000 Greece AND has_point_prevalence_average_value : 36.5 AND has_point_prevalence_range : 1-5 / 10 000 Brunei Darussalam AND has_point_prevalence_average_value : 2.6 AND has_point_prevalence_range : 1-9 / 100 000 France AND has_annual_incidence_average_value : 0.9 AND has_annual_incidence_range : 1-9 / 1 000 000 Israel AND has_point_prevalence_average_value : 5.5 AND has_point_prevalence_range : 1-9 / 100 000 Greece AND has_annual_incidence_average_value : 2.1 AND has_annual_incidence_range : 1-9 / 100 000 Australia AND has_point_prevalence_average_value : 5.1 AND has_point_prevalence_range : 1-9 / 100 000 Worldwide AND has_point_prevalence_average_value : 21.05 AND has_point_prevalence_range : 1-5 / 10 000 Denmark AND has_point_prevalence_average_value : 12.0 AND has_point_prevalence_range : 1-5 / 10 000 Europe AND has_point_prevalence_average_value : 25.0 AND has_point_prevalence_range : 1-5 / 10 000 |
|
treeView | ||
subClassOf |