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Primary Biliary Cirrhosis Mortality
Primary biliary cirrhosis is a rare form of liver disease (cirrhosis) which results from the destruction of the bile ducts within the liver. The cause of primary biliary cirrhosis is unknown. The liver destruction is thought to occur on the basis of an autoimmune mechanism where there is production of antibodies which attack specific portions of the liver. Genetic factors may also be important since the disease is seen with increased frequency in the offspring of affected mothers. Females are affected more commonly with approximately 90% of all cases of primary biliary cirrhosis in females. This disease has been diagnosed in patients as young as 23 years and as old as 72. The majority of cases are diagnosed in the 40-60 age group. The most common presenting symptom is itching. This may occur after the onset of birth control pill use or during pregnancy. Jaundice (yellowing of the eyes, skin, and under the tongue) is seen as a later finding. Advanced disease leads to all the findings and serious complications which typify cirrhosis from other causes. Laboratory findings include elevations in the blood cholesterol and alkaline phosphatase levels. Some mild elevation may be seen in the transaminase liver enzymes. Special serologic tests (anti-mitochondrial antibodies) help confirm the diagnosis. Liver biopsy provides absolute confirmation for this diagnosis. There is no specific treatment for primary biliary cirrhosis. Treatment largely consists of supportive care for cirrhosis and it's complications. These patients benefit from treatment of high cholesterol and itching with medications (cholestyramine). Calcium supplementation and vitamin D can also be effective. A diet that is low in fat (40 grams daily) is recommended. Alcoholic beverages must be avoided. Other medications that are primarily metabolized (and eliminated) by the liver will require dosage adjustment (acetaminophen). Patients who are without symptoms at the time of diagnosis (picked up on routine blood tests) have a better prognosis and can live 10 years or more, often without symptoms. Those who manifest symptoms of this disease, in contrast, only have a 50% survival rate beyond 5 years. The internist or gastroenterologist are the experts in the diagnosis and management of this serious disorder.
Hope this article will provide you information about primary biliary cirrhosis.
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