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Primary Biliary Cirrhosis

Definition: A chronic disease of the liver with slow, progressive damage to the bile ducts that interferes the excretion of bile from the liver. In its early stages, substances that are normally eliminated into the bile, such as acids and cholesterol, accumulate in the blood presenting complications. Primary biliary cirrhosis causes continued inflammation of the liver, which in turn causes scarring leading to cirrhosis in the later stage.

Symptoms: Patients often have no symptoms and are usually diagnosed after showing abnormalities on routine liver blood tests. The disease is mainly diagnosed in people between 30 and 60 years of age, affecting women 10 times more often than men. The most common symptoms are itching and fatigue, jaundice, acummulation of fluid, darkening of the skin and fatty deposits in the skin are also indications of the disease. Primary biliary cirrhosis can cause various disorders, which commonly include impairment of the salivary and tear glands causing dry mouth and eyes. Other problems it may present are arthritis and thyroid problems, and at late stage, the possibility of fractures due to soft and fragile bones.

Although primary biliary cirrhosis is not directly inherited, it is more common among siblings and families in which at least one member has been affected. Another important factor may be multiple immune system disorders found in patients. Also, the fact that this disease is much more common in women brings hormones to issue.

Diagnosis: Several pieces of information are considered at the moment of diagnosis. An alkaline phosphatase activity may confirm bile duct damage. Yet, due to the possibility of obstruction, oftentimes an x-ray is taken to rule out this cause. Mitochondrial antibody tests are positive in almost all individuals with primary biliary chirrhosis and a liver biopsy would provide proof on the extent and severity of damage to the liver, thus confirming the diagnosis. Still, the basic cause of primary biliary cirrhosis is unknown, although close observation of patients over the years has provided many clues and has lead to earlier diagnosis.

Prognosis: Known for over 100 years, primary biliary cirrhosis is a slowly progressing disease. Many patients can expect to lead productive and active lives for up to fifteen years after they are diagnosed and may not have symptoms for several years. As the disease progresses, jaundice may appear as sign of diminishing liver reserve. This is a chronic illness often presenting life-threatening complications after it advances to cirrhosis.

Treatment and The Alternative of Transplantation: Some drugs such as methotrexate and ursodeoxycholic acid have shown improvement in liver tests, but most patients do no respond to corticosteroids. Colchicine also improves liver tests, although it does not slow the progression of the disease. Thyroid function tests to determine thyroid activity should be performed and if low, hormone treatment prescribed. Care should be taken with potentially harmful drugs, toxins or foods. Itching can be effectively relieved by medications such as rifampin and cholestryramine.

Finally, a liver transplant may be indicated before the onset of severe liver failure and related complications such as gastrointestinal bleeding, malnutrition, accumulation of fluid in the abdomen, jaundice, persistent itching and bone fractures. Patients with primary biliary cirrhosis who have undergone liver transplant have excellent outcome and survival rates, with the use of new anti-rejection drugs making it even more successful.

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