Vanishing bile duct syndrome
Vanishing Bile Duct Syndrome
Vanishing bile duct syndrome (pronunciation: van·ish·ing bile duct syn·drome) is a rare, progressive disease characterized by the destruction and disappearance of the bile ducts within the liver. The etymology of the term is derived from the English words "vanishing" (disappearing), "bile" (a yellow-green fluid produced by the liver), and "duct" (a tube or passageway), and "syndrome" (a group of symptoms that consistently occur together).
Definition
Vanishing bile duct syndrome, also known as ductopenia, is a condition where the bile ducts, which are necessary for the transport of bile from the liver to the gallbladder and intestines, progressively disappear. This leads to a buildup of bile in the liver, causing jaundice, itching, and eventually liver failure.
Causes
The exact cause of vanishing bile duct syndrome is unknown. However, it has been associated with various conditions such as autoimmune diseases, infections, adverse drug reactions, and malignancies.
Symptoms
The symptoms of vanishing bile duct syndrome include jaundice, severe itching, fatigue, weight loss, and abdominal pain. As the disease progresses, it can lead to complications such as cirrhosis and liver failure.
Diagnosis
Diagnosis of vanishing bile duct syndrome is typically made through a combination of clinical symptoms, blood tests, imaging studies, and liver biopsy. The definitive diagnosis is made by a liver biopsy showing loss of bile ducts.
Treatment
Treatment of vanishing bile duct syndrome is primarily supportive and aimed at managing symptoms. This may include medications to reduce itching, vitamin supplements to replace deficiencies caused by malabsorption, and in severe cases, liver transplantation.
See Also
External links
- Medical encyclopedia article on Vanishing bile duct syndrome
- Wikipedia's article - Vanishing bile duct syndrome
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