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Digestive system
Digestive system


Bile pathway
Bile pathway


Definition:

Primary biliary cirrhosis is irritation and swelling (inflammation) of the bile ducts of the liver, which blocks the flow of bile . This obstruction damages liver cells.



Alternative Names:

PBC



Causes, incidence, and risk factors:

The cause of inflamed bile ducts in the liver is not known. The disease more commonly affects middle-aged women.

Long-term bile obstruction is believed to lead to liver cirrhosis . The disease may be associated with autoimmune disorders such as:



Symptoms:

Symptoms usually come on gradually and may include:



Signs and tests:

Tests for the disease:

Tests for liver dysfunction :



Treatment:

Therapy aims to relieve symptoms and prevent complications.

Cholestyramine (or colestipol) may reduce the itching. Ursodeoxycholic acid may improve removal of bile from the bloodstream.

Vitamin replacement therapy restores vitamins A, K, and D, which are lost in fatty stools. A calcium supplement can help prevent soft, weakened bones (osteomalacia ).

Liver transplant before liver failure occurs may be successful.



Support Groups:



Expectations (prognosis):

The outcome can vary. If the condition is not treated, most patients will need a liver transplant after about 7 years. Doctors can now use statistics to predict the best time to do the transplant.



Complications:

Progressive cirrhosis can lead to liver failure. Complications can include:



Calling your health care provider:

Call your health care provider if you have:



Prevention:



References:

Silveira MG, Lindor KD. Treatment of primary biliary cirrhosis: therapy with choleretic and immunosuppressive agents. Clin Liver Dis. 2008;12:425-443.

Mayo MJ. Natural history of primary biliary cirrhosis. Clin Liver Dis. 2008;12:277-288.




Review Date: 5/20/2008
Reviewed By: Christian Stone, MD, Division of Gastroenterology, Washington University in St. Louis School of Medicine, St. Louis, MO. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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