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Primary biliary cirrhosis (CUI C0008312) Suggest changes to this concept
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Terms & Properties

Concept Unique Identifier (CUI): C0008312

NCI Thesaurus Code: C27167  (see NCI Thesaurus info)

Semantic Type: Disease or Syndrome

NCIt Definition: 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.

GARD Definition: Primary biliary cirrhosis is a chronic disease that affects the bile ducts within the liver. The bile ducts become inflamed and damaged, which causes bile to build up in the liver. This abnormal buildup destroys liver tissue and results in cirrhosis. Primary biliary cirrhosis usually occurs between the ages of 40 and 60 and affects women more often than men. The cause of this condition is unknown, but research suggests that it is an autoimmune diseaseUrsodiol is the only drug approved by the U.S. Food and Drug Administration for the treatment of primary biliary cirrhosis. - this information is from GARD/ORDR/NCATS.

CSP Definition: 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.

Synonyms & Abbreviations: (see Synonym Details)
Biliary cirrhosis primary
Biliary Cirrhosis, Primary, 1
Biliary Cirrhosis, Primary
Cholangitis, Chronic Nonsuppurative Destructive
Chronic non-suppurative destructive cholangitis
Chronic nonsuppurative destructive cholangitis
CIRRHOSIS, PRIMARY BILIARY
PBC1
PBC
Primäre biliäre Zirrhose
Primary Bilary Cirrhosis (PBC)
Primary biliary cholangitis (disorder)
Primary biliary cholangitis
PRIMARY BILIARY CIRRHOSIS

External Source Codes: 
NCI Thesaurus Code C27167 (see NCI Thesaurus info)

Other Properties: Property Definitions
Name Value Source
ACTIVE 1 SNOMEDCT_US
CASE_SIGNIFICANCE_ID 900000000000020002 SNOMEDCT_US
CTV3ID J6160 SNOMEDCT_US
DATE_CREATED 2013-01-09T00:04:00 GARD
DATE_LAST_MODIFIED 10/09/2014 RADLEX
DATE_LAST_MODIFIED 2013-01-09T00:04:00 GARD
DEFINITION_STATUS_ID 900000000000074008 SNOMEDCT_US
DID 4000-0298 CSP
DISEASE_IDENTIFIER_ID 5480 GARD
EFFECTIVE_TIME 20020131 SNOMEDCT_US
GENELOCUS ,3,p,1,2,-,q,1,3,.,2, OMIM
GENESYMBOL PBC1 OMIM
HAS_GARD_PAGE true GARD
IAN DEFAULT ICD10
IDENTIFIER_TYPE_ID 1 GARD
INCLUSION_TERM Chronic nonsuppurative destructive cholangitis ICD10
IS_ACTIVE true GARD
IS_RARE true GARD
IS_SPANISH false GARD
LAST_CHANGES_TO_CLASS syn RADLEX
LAST_REVIEW_DATE 2008-10-14T00:00:00 GARD
MIMTYPE 5 OMIM
MIMTYPEMEANING Mendelian phenotype or locus, molecular basis unknown. OMIM
MIMTYPEVALUE perc OMIM
ORDER_NO 13576 ICD10CM
PRIMARY_PATH 10004661$10019669$10019654$10019805$Biliary cirrhosis primary$Hepatic fibrosis and cirrhosis$Hepatic and hepatobiliary disorders$Hepatobiliary disorders MDR
PRIMARY_SOC 10019805 MDR
PT_IN_VERSION 10.0 MDR
PT_IN_VERSION 10.1 MDR
PT_IN_VERSION 11.0 MDR
PT_IN_VERSION 11.1 MDR
PT_IN_VERSION 12.0 MDR
PT_IN_VERSION 12.1 MDR
PT_IN_VERSION 13.0 MDR
PT_IN_VERSION 13.1 MDR
PT_IN_VERSION 14.0 MDR
PT_IN_VERSION 14.1 MDR
PT_IN_VERSION 15.0 MDR
PT_IN_VERSION 15.1 MDR
PT_IN_VERSION 16.0 MDR
PT_IN_VERSION 16.1 MDR
PT_IN_VERSION 17.0 MDR
PT_IN_VERSION 17.1 MDR
PT_IN_VERSION 18.0 MDR
PT_IN_VERSION 18.1 MDR
PT_IN_VERSION 19.0 MDR
PT_IN_VERSION 19.1 MDR
PT_IN_VERSION 8.0 MDR
PT_IN_VERSION 8.1 MDR
PT_IN_VERSION 9.0 MDR
PT_IN_VERSION 9.1 MDR
RARE_DISEASE_URL http://rarediseases.info.nih.gov/gard/7459/primary-biliary-cirrhosis/resources/1 GARD
SMQ_TERM_ADDVERSION 10.1 MDR
SMQ_TERM_ADDVERSION 8.0 MDR
SMQ_TERM_CAT A MDR
SMQ_TERM_LEVEL 4 MDR
SMQ_TERM_LEVEL 5 MDR
SMQ_TERM_LMVERSION 12.1 MDR
SMQ_TERM_LMVERSION 16.0 MDR
SMQ_TERM_SCOPE 2 MDR
SMQ_TERM_STATUS A MDR
SMQ_TERM_WEIGHT 0 MDR
SNOMEDID D5-80720 SNOMEDCT_US
SUBSET_MEMBER 447562003~CORRELATIONID~447561005 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPADVICE~ALWAYS K74.3 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPCATEGORYID~447637006 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPGROUP~1 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPPRIORITY~1 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPRULE~TRUE SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPTARGET~K74.3 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~CORRELATIONID~447561005 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~ALWAYS K74.3 | CONSIDER ADDITIONAL CODE TO IDENTIFY SPECIFIC CONDITION OR DISEASE SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPCATEGORYID~447637006 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPGROUP~1 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPPRIORITY~1 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPRULE~TRUE SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPTARGET~K74.3 SNOMEDCT_US
SUBSET_MEMBER 900000000000497000~MAPTARGET~J6160 SNOMEDCT_US
SUBSET_MEMBER 900000000000498005~MAPTARGET~D5-80720 SNOMEDCT_US
SUBSET_MEMBER 900000000000508004~ACCEPTABILITYID~900000000000548007 SNOMEDCT_US
SUBSET_MEMBER 900000000000508004~ACCEPTABILITYID~900000000000549004 SNOMEDCT_US
SUBSET_MEMBER 900000000000509007~ACCEPTABILITYID~900000000000548007 SNOMEDCT_US
SUBSET_MEMBER 900000000000509007~ACCEPTABILITYID~900000000000549004 SNOMEDCT_US
TERMUI T024190 MSH
TERMUI T521207 MSH
TERMUI T526174 MSH
TERMUI T811452 MSH
TH NLM (2004) MSH
TH OMIM (2013) MSH
TH ORD (2010) MSH
TH UNK (19XX) MSH
TYPE_ID 900000000000003001 SNOMEDCT_US
TYPE_ID 900000000000013009 SNOMEDCT_US

Additional Concept Data:  (none)

URL to Bookmark: https://ncim-stage.nci.nih.gov/ncimbrowser/ConceptReport.jsp?dictionary=NCI Metathesaurus&code=C0008312

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