Primary biliary cirrhosis: Primary biliary cirrhosis is a liver disease that slowly destroys the bile ducts in the liver. Bile, a substance that helps digest fat, leaves the liver ... more about Primary biliary cirrhosis.
Primary biliary cirrhosis: Primary biliary cirrhosis is a chronic and progressive cholestatic disease of the liver. More detailed information about the symptoms, causes, and treatments of Primary biliary cirrhosis is available below.
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Primary biliary cirrhosis is a liver disease that slowly destroys the bile ducts in the liver. Bile, a substance that helps digest fat, leaves the liver through these ducts. When the ducts are damaged, bile builds up in the liver and damages liver tissue. Over time, the disease can cause cirrhosis and may make the liver stop working. (Source: excerpt from Primary Biliary Cirrhosis: NIDDK)
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. - (Source - Diseases Database)
Primary biliary cirrhosis is listed as a "rare disease" by the Office of
Rare Diseases (ORD) of the National Institutes of Health
(NIH). This means that Primary biliary cirrhosis, or a subtype of Primary biliary cirrhosis,
affects less than 200,000 people in the US population.
Source - National Institutes of Health (NIH)
Ophanet, a consortium of European partners,
currently defines a condition rare when it affects 1 person per 2,000.
They list Primary biliary cirrhosis as a "rare disease".
Source - Orphanet
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