24October2017

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Biliary atresia- Drug Today Medical Times

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Biliary atresia is a rare disease of the liver and bile ducts that occurs in newborns. Biliary atresia is occuring more in East Asia, with frequency of one in 5,000. The disease is also known as extrahepatic dectopaenia and progressive obliterative cholangiopathy. Problems such as ducts are abnormally narrow, blocked or absent, are seen.

It can be congenital or acquired. As a birth defect in newborn infants, it has incidence of one in 10,000-15,000 live births in the United States and prevalence of one in 16,700 in British Isles.

Symptoms of the disease appear or develop about two to eight weeks after birth. In the beginning, the symptoms of this rare disease are indistinguishable from those of neonatal jaundice, as happens in infants. Distinctive 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 not able 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 skin, itchiness, poor absorption of nutrients (causing delays in growth), pale stools, dark urine, and a swollen abdomen. Around 90 per cent of infants with biliary atresia will have to undergo an operation designed to retain and salvage the native liver, restore bile flow and reduce the level of jaundice. This procedure is known as Kasai procedure.