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Biliary atresia
Biliary atresia, also known as "extrahepatic ductopenia" and "progressive obliterative cholangiopathy", is a childhood disease of the liver in which one or more bile ducts are abnormally narrow, blocked, or absent. It can occur as a birth defect or as an acquired disease. As a birth defect in newborn infants, it has an incidence of one in 10,000 to 15,000 cases in live births in the United States, with the most accurate prevalence recorded at one in 16,700 in the British Isles. Biliary atresia is most common in East Asia, with a frequency of one in 5,000. In the congenital form, the common bile duct between the liver and the small intestine is either blocked or absent. The causes of biliary atresia are not well understood. Congenital biliary atresia has been associated with certain genes, while acquired biliary atresia is thought to be the result of an autoimmune inflammatory response, possibly to a viral infection of the liver soon after birth. The only effective treatments are certain surgeries such as the Kasai procedure and liver transplantation.
==Signs and symptoms==
Initially, the symptoms of biliary atresia are indistinguishable from neonatal jaundice, a usually harmless condition commonly seen in infants after birth. Symptoms of biliary atresia are usually evident between one and six weeks after birth. Infants and children with biliary atresia develop progressive cholestasis, a condition in which bile is unable to leave the liver and builds up inside of it. When the liver is unable to excrete bilirubin through the bile ducts in the form of bile, bilirubin begins to accumulate in the blood, causing symptoms. These symptoms include yellowing of the skin, itchiness, poor absorption of nutrients (causing delays in growth), pale stools, dark urine, and a swollen abdomen. Eventually cirrhosis with portal hypertension will develop. Left untreated, biliary atresia can lead to liver failure. Unlike other forms of liver failure, however, biliary atresia-related liver failure does not result in kernicterus, a form of brain damage resulting from liver dysfunction. The reason for this is that the liver, although diseased, is still able to conjugate bilirubin, and conjugated bilirubin is unable to cross the blood–brain barrier.
抄文引用元・出典: フリー百科事典『 ウィキペディア（Wikipedia）』
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