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Primary biliary cirrhosis Causes and Risks:
Bile ducts within the liver become inflamed due to unknown cause. The disease affects middle-aged women most frequently. The onset of symptoms is gradual, with itching skin as the first symptom. Inflammation of the bile ducts within the liver occurs. Eventually, liver cirrhosis develops. The disease may be associated with autoimmune disorders . The incidence is 8 out of 100,000 people.
Prevention:
There is no known prevention.
Symptoms:
Signs and Tests:
Tests that confirm cirrhosis : Tests that reveal liver dysfunction : Serum cholesterol and lipoproteins may be increased.
This disease may also alter the results of the following tests:
Treatment:
Therapy is aimed at symptomatic treatment and prevention of complications.
Cholestyramine or colestipol may reduce the itching by reducing blood levels of bile acids, and clearance of bile acids in the skin. Vitamin A , vitamin K , and vitamin D replacement therapy adds fat-soluble vitamins lost with fatty stools. A calcium supplement is indicated to prevent osteomalacia .
Liver transplant for advanced primary biliary cirrhosis prior to liver failure has been successful in some patients.
Prognosis:
The course of the disease varies; slow progression is associated with prolonged survival in some people. Untreated, cirrhosis leads to death.
Complications:
Progressive cirrhosis can lead to bleeding in the esophagus, hypertension in the veins of the liver, and encephalopathy (brain involvement). Osteomalacia , a condition that softens the bones, may develop.
Call Your Healthcare Provider:
Call for an appointment with your health care provider if prolonged itching of the skin develops that is not related to other causes, or if jaundice or other symptoms of cirrhosis develop.
Vomiting blood , blood in the stools , confusion , and decreased mental states are symptoms that indicate cirrhosis may be worsening. Call your health care provider if these or other new symptoms develop.
This illustration shows the major organs of the digestive system. Their relative sizes and positioning in the body are also demonstrated.
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