Xanthomatous biliary cirrhosis

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Xanthomatous Biliary Cirrhosis

Xanthomatous Biliary Cirrhosis (pronunciation: zan-tho-ma-tous bil-iary sir-roh-sis) is a rare form of liver disease characterized by the accumulation of xanthoma cells in the biliary tract.

Etymology

The term 'Xanthomatous' is derived from the Greek words 'xanthos' meaning yellow and 'oma' meaning tumor, referring to the yellowish nodules formed by the accumulation of xanthoma cells. 'Biliary' refers to the biliary tract, the pathway by which bile is transported from the liver to the small intestine. 'Cirrhosis' is derived from the Greek word 'kirrhos' meaning tawny, referring to the orange-yellow color of the diseased liver.

Definition

Xanthomatous Biliary Cirrhosis is a chronic and progressive liver disease where the bile ducts in the liver are gradually destroyed. The destruction of the bile ducts leads to a buildup of bile in the liver, causing damage and scarring (cirrhosis). This condition is often associated with the presence of xanthoma cells, which are lipid-laden macrophages that can accumulate in various organs, including the liver.

Symptoms

The symptoms of Xanthomatous Biliary Cirrhosis can vary, but often include jaundice, itching, fatigue, and abdominal pain. As the disease progresses, it can lead to complications such as portal hypertension, liver failure, and an increased risk of liver cancer.

Treatment

Treatment for Xanthomatous Biliary Cirrhosis primarily involves managing the symptoms and slowing the progression of the disease. This can include medications to reduce itching and improve bile flow, vitamin supplements to address malnutrition, and in severe cases, a liver transplant may be necessary.

Related Terms

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