An uncommon form of liver cirrhosis that results from problems with the bile ducts, either due to an autoimmune disorder known as primary biliary cirrhosis, or a longstanding blockage. Primary biliary cirrhosis affects mainly middle-aged women and seems to be linked with a malfunction of the immune system. Secondary biliary cirrhosis results from prolonged bile duct obstruction or biliary atresia. In both types, liver function is impaired due to cholestasis (accumulation of bile in the liver). In primary biliary cirrhosis, the bile ducts within the liver become inflamed and are destroyed. Symptoms include itching, jaundice, an enlarged liver, and sometimes abdominal pain, fatty diarrhoea, and xanthomatosis. Osteoporosis may develop. Symptoms of liver cirrhosis and liver failure may occur after several years. Drugs can minimize complications and relieve symptoms such as itching. A liver transplant is the only long-term cure.
The symptoms and signs of secondary biliary cirrhosis include abdominal pain and tenderness, liver enlargement, fevers and chills, and sometimes blood abnormalities. Treatment is the same as for bile duct obstruction.
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