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"Primary biliary cirrhosis" information

LiverAtlas Disease IDHuLDi00112
NamePrimary biliary cirrhosis
Description Xref:
Definition1.An autoimmune inflammatory disorder characterized by destruction of the small intrahepatic bile ducts. It affects predominantly females and it may lead to cirrhosis and liver failure. Patients have antimitochondrial and antinuclear antibodies in the peripheral blood. Xref: UMLS2011AA:C0008312
2.A form of biliary cirrhosis in which small intrahepatic ducts are destroyed while the major intra and extra hepatic ducts remain patent; most patients are middle aged females and have circulating antimitochondrial antibodies. Xref: UMLS2011AA:C0008312
SynonymBiliary Cirrhosis, PrimaryEXACT Xref: MeSH2011_6_17:D008105
PBCEXACT Xref: Clinical Hepatology, 2004:Page 437
primary biliary cirrhosisEXACT Xref: CSP2005:4000-0298, HumanDO2011_6_18:DOID:12236
Primary biliary cirrhosisEXACT Xref: UMLS2011AA:C0008312
Primary Biliary CirrhosisEXACT Xref: Schiff's Diseases of the LIVER, 10ed:Chapter 24, MeSH2011_6_17:D008105
XrefClinical Hepatology,2004.Page 483
HumanDO2011_6_18:DOID:12236
MeSH2011_6_17:D008105
Genetics RelationGene/Protein/Pathway
Gene:ALB
Gene:ALPP
Gene:AIRE
Gene:FAS
Gene:FAS
Gene:ASGR2
Gene:BCL2
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