Understanding Cirrhosis of the Liver
What Causes Cirrhosis of the Liver? continued...
Cirrhosis sometimes, though rarely, occurs because of an inherited liver disorder. In Wilson's disease, for example, a genetic deficiency inhibits the body's ability to metabolize copper. As a result, excessive amounts of the metal accumulate in various body organs, particularly the liver, where it destroys tissue. Similarly, in hemochromatosis the body absorbs excess amounts of iron, which damages the liver and causes scarring. This disorder mostly strikes men between the ages of 40 and 60; women who have not gone through menopause are usually not affected because their bodies lose iron during menstruation. Alpha-1-antitrypsin deficiency is an enzyme deficiency that results in the accumulation of products in the liver causing destruction of liver tissue.
Children born with galactosemia lack an enzyme needed to digest a component of milk sugar. Milk sugar, also known as lactose, is composed of two sugars, glucose and galactose. The body needs to convert the galactose to glucose. In people with galactosemia, the enzyme to do this conversion is missing or not functioning adequately. Galactose accumulates in the liver at levels that become toxic and potentially fatal without proper treatment. Infants with this disorder should be taken off milk and given a substitute formula that does not contain galactose.
Some babies are born with no bile ducts, or with ducts that are malformed. Because bile is unable to drain out of the body, it accumulates in the liver and eventually poisons it. Although the problem can sometimes be corrected through surgery, most children with this disorder die from cirrhosis before they reach the age of 2.
Cirrhosis can result when strictures or scarring block the flow of bile in the bile ducts and cause it to back up into the liver for long periods of time. This occurs in conditions such as primary sclerosing cholangitis or primary biliary cirrhosis. The disease may also come as a consequence of long-term exposure to certain drugs, including methotrexate and isoniazid, and to toxic substances in the environment, such as pesticides and arsenic-based compounds. Lastly, autoimmune hepatitis is an inflammatory process in the liver that can result in scarring and cirrhosis due to antibodies produced by the body that attack the liver. The cause is unknown.