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Primary biliary cirrhosis (PBC) definition
Primary biliary cirrhosis (PBC) is a progressive disease of the liver caused
by a buildup of bile within the liver (cholestasis) that results in damage to
the small bile ducts that drain bile from the liver. Over time, this pressure
build-up destroys the bile ducts leading to liver cell damage. As the disease
progresses and enough liver cells die,
cirrhosis and liver failure occur.
Bile is manufactured in the liver and then transported through the bile ducts
to the gallbladder and intestine where it helps digest fats and fat soluble
vitamins (A,D,E,K). When bile cannot be drained from the liver, it causes
inflammation that leads to cell death. Scar tissue gradually replaces the areas
of damaged liver and then the body cannot perform necessary functions.