
Treatment of primary biliary cirrhosis includes correction of nutritional abnormalities. Patients may develop vitamin deficiencies. Since the deficiencies are usually associated with fat-soluble vitamins, repletion with vitamins A, D, E and K is necessary.
Elevated levels of cholesterol may also be associated with PBC. In fact this is a rather common finding. It is most marked in the early part of the disease.
Medications that lower lipids and cholesterol are appropriate for these individuals. The elevated cholesterol accounts for the fatty deposits noted around the eyes (xanthelasma).
Cholestyramine may be utilized for some of these changes as well.
Ursochenodeoxycholic acid (bile acid therapy) may be extremely useful. In fact, in most cases, this is as effective as a single agent.
Other medications such as aziothioprine (or 6-mercaptopurine), steroids, cyclosporin as well as methotrexate are less effective. In some cases, antifibrotic (compounds which help prevent early scarring of the liver) agents such as colchicine may be used.
The natural history of the disease is one of aggressive development until liver cirrhosis has become established. Many patients do require liver transplantation for their disease.
Complications of PBC
Complications of PBC include:
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Osteopenia |
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Hypercholesterolemia |
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Fat soluble vitamin deficiencies |
Complications of Cholestatic Diseases
Complications of cholestatic diseases include:
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PBC |
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Primary sclerosing cholangitis |
Both of these disorders are associated with chronic elevations in the liver function tests. This is predominantly characterized by an elevation in the alkaline phosphatase.
Complications of any cholestatic disorder include:
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Osteopenia |
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Hypercholesterolemia |
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Fat soluble vitamin deficiencies |
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Copper accumulation |
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Alcoholic Liver Disease |
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Iron Overload Diseases |
Primary Biliary Cholangitis |
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