What does biliary atresia mean?

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Biliary-Atresia

Biliary atresia is a rare disease that occurs in infants. It affects the bile ducts and the liver.
Bile is a liquid produced by cells inside the liver. Bile is useful in fat digestion and for carrying waste products from the liver, to the intestines for the purpose of excretion. The biliary system is the network of ducts and channels and when it functions properly, it allows the bile to drain into the intestines from the liver.

Not much is understood about causes of this disease. However research indicates that in pregnancy, the bile ducts do not form properly and could cause Biliary Atresia. In other cases, immune system of the body could get damaged by the bile ducts in response to a viral infection acquired after the infant’s birth.

This disorder is a rare one. Around 15000 to 20000 babies have a rare disorder of not having complete bile ducts. More than boys, girls are affected more by Biliary Atresia. If there is one pair of twins then one child could get the disease. In certain cases one child within the same family could get the disease. As compared to Caucasians, chances of African Americans and Asians of getting the disease are more.

How is biliary atresia diagnosed?

An infant born with Biliary Atresia disease could develop problems in the spleen, intestine, heart and blood vessels. When a infant affected with Biliary Atresia is born, it is healthy usually. However within about 2-8 weeks, symptoms of the disease develop or appear.

Symptoms of Biliary Atresia disease include jaundice, a yellow coloring of the eyes and skin, alcoholic or clay colored stools, dark urine, irritability and loss of weight.

Disorders in the liver are generally linked with jaundice. Hence to attain a correct diagnosis, several tests have to be conducted. Blood tests are done, abdominal ultrasound is done and X-Rays of the abdomen are taken. In some cases a biopsy of the liver, diagnostic surgery or an operative cholangiogram is done.

It is difficult to treat Biliary Atresia with medication. However a Hepatoportoenterostomy or Kasai procedure is done. This operation is done by an experienced surgeon to restore flow of bile to the intestine from the liver. A final option is a liver transplantation, in case the Kasai procedure fails. More calories including a well balanced diet, vitamin supplements, also may have to be given to children suffering with Biliary Atresia.