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  • Biliary Atresia

    Biliary atresia is a condition found exclusively in infants that causes the bile ducts become blocked or inflamed. This prevents the secretion of bile into the digestive tract and causes life-threatening liver damage. Bile is digestive fluid necessary to digest fatty acids and vitamins.

    There are two types of biliary atresia: fetal, when it appears in babies still in the womb, and perinatal, which is discovered weeks after the child is born. Perinatal is the more common form of the disease. The disease affects just 1 in approximately 18,000. Girls, those born premature and those with Asian or African heritage are most susceptible.

    Causes

    The cause of biliary atresia is unknown. However, researchers know the condition is neither hereditary nor contagious. The mother’s lifestyle during pregnancy is not believed to affect whether the child contracts the disease.

    The most likely causes of biliary atresia are:
    • Developmental issues with the liver and/or bile duct in utero
    • Immune system defects
    • Genetic mutations
    • An after-birth infection
    • Contact with toxic materials

    Symptoms

    Jaundice, or the yellowing of the skin and eyes is the primary symptom. Triggered by the body’s inability to properly release bile into the digestive tract, bilirubin, the substance found in the blood stream that the liver absorbs to produce bile, builds up into the blood stream and causes the discoloration.

    Other symptoms may include:
    • Abdominal swelling
    • Hardening of the liver
    • Darkened urine – this is due to the saturation of bilirubin into the blood stream
    • Pale or colorless stool – due to the lack of bile in the intestinal tract

    Testing and Diagnosis

    There is not a test to successfully determine whether a child has biliary atresia. Instead, doctors must use a battery of tests and procedures to determine the child’s condition. They include:
    • Blood tests for the amount of bilirubin
    • X-rays
    • Scans and ultrasounds
    • Biopsy
    • Exploratory surgery

    Treatment

    One of two surgical procedures is required to treat biliary atresia, either a liver transplant or the Kasai procedure.

    With a liver transplant, the child can receive either a complete liver from a recently deceased child or a partial liver from either a deceased or living adult. If received from an adult, the healthy tissue will develop into a full liver during the child’s developmental years.

    The Kasai procedure is named after the surgeon who developed the technique. During the procedure, the surgeon removes the damaged bile ducts. It their place, the surgeon takes a piece of the infant’s intestinal tract and connects it to the liver. This gives bile a direct conduit to the digestive system.

    The Kasai procedure typically is most effective in children less than 12 weeks old who have not yet suffered permanent liver damage. The treatment does not cure the disease, but it does increase the chances for a normal, healthy life.

    In cases of fetal biliary atresia, a liver transplant is the preferred treatment as the damage occurs earlier in development.



Page last updated: 1/6/2014 5:16:17 PM