Primary Biliary Cirrhosis (PBC) (cont.)

Elevated serum cholesterol and xanthomas

Elevated levels of cholesterol in the blood are common in patients with PBC, and xanthomas (fatty deposits that appear as yellowish firm nodules in the skin) occur in about 25% of those patients with elevated cholesterol. Diets with low cholesterol content do not consistently lower serum cholesterol in these patients, because production of cholesterol by the liver is stimulated in patients with PBC. Cholestyramine, the oral medication that is often used to treat itching, can, at the same time, reduce the levels of serum cholesterol to a modest degree.

Clofibrate (Atromid) should not be used for treating elevated serum cholesterol in PBC because it elevates (rather than lowers) the cholesterol levels in these patients. Moreover, this drug may worsen xanthomas and cause formation of gallstones containing cholesterol. Two studies indicate that UDCA therapy significantly reduces serum levels of cholesterol and is recommended for use in patients with xanthomas. A new class of drugs called statins inhibits formation of cholesterol and, to a lesser degree, triglycerides. The safety and effectiveness of the statins, however, have not been adequately studied in PBC. One of the common side effects of statins is liver injury. Thus, their use in a person with liver disease requires careful monitoring by a physician.

Malabsorption of fat and fat-soluble vitamins (A, D, E and K)

Reduction of dietary fat is the treatment of choice for fat malabsorption (poor absorption of fat in the gut). The idea is that if the dietary intake of fat is decreased, more of this fat will be absorbed. The goal of the low-fat diet would be to alleviate the diarrhea caused by the fat malabsorption, while still providing enough fat for adequate nutrition. If this diet does not help, a supplement of special fats called medium-chain triglycerides (MCT) can be ingested. MCT can replace as much as 60% of the calories provided by ordinary dietary fat, which is mostly long-chain triglycerides. MCT is a special type of fat preparation that does not require bile acids for its absorption and is actually absorbed more easily than the usual dietary fat. As noted earlier, PBC patients with malabsorption of fat should also be tested for celiac sprue.

It is recommended that patients with PBC take a multivitamin supplement without minerals to increase the dietary intake of fat-soluble vitamins. If the quantities of bile acids flowing through the bile ducts to the gut are marginal, intestinal absorption of the fat-soluble vitamins may not be adequate, even with supplements. Two strategies exist for this situation. First, patients can take Liqui-E with meals. Liqui-E is an over-the-counter liquid preparation of vitamin E that also increases the absorption of other fat-soluble vitamins in the diet or in multivitamin preparations. Second, the fat-soluble vitamins A and K can be given by injection into the muscle once a month. Remember, however, that women who might become pregnant, should not receive injections of vitamin A, because it can cause birth defects.



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