Biliary atresia seems to affect girls slightly more often than boys. Asians and African-Americans are affected more frequently than Caucasians. There does not appear to be any link to medications or immunizations given immediately before or during pregnancy.
Biliary Atresia is a rare gastrointestinal disorder. Biliary atresia is characterized by obliteration or discontinuity of the extrahepatic biliary system, resulting in obstruction to bile flow. Bile is a yellow-green fluid made in the liver and stored in the gallbladder. The disorder represents the most common surgically treatable cause of cholestasis encountered during the newborn period. About one in 10,000 to 20,000 babies in the U.S are affected every year.
Symptoms of the disease appear or develop about two to eight weeks after birth. Symptoms of the disease include Jaundice a yellow coloring of the skin and eyes due to a very high level of bilirubin (bile pigment) in the bloodstream.Jaundice caused by an immature liver is common in newborns. It usually goes away within the first week to 10 days of life. Dark urine caused by the build-up of bilirubin (a breakdown product from hemoglobin) in the blood. The bilirubin is then filtered by the kidney and removed in the urine.
Weight loss and irritability develop when the level of jaundice increases. Extrahepatic biliary atresia occurs more commonly in females than in males. Biliary atresia causes liver damage and affects numerous important processes that allow the body to function normally. Biliary atresia is an irreversible problem. There are no medications that can be given to unblock the bile ducts or to encourage new bile ducts to grow where there were none before. Two different operations can be done that will allow the child with biliary atresia to live longer and have a better quality of life.
Kasai portoenterostomy operation connects the bile drainage from the liver directly to the intestinal tract. It is most successful when done before an babys is 8 weeks old. The Kasai procedure is helpful because it can allow a child to grow and wait in fairly good health for several years. Cirrhosis, or scarring of the liver, generally occurs despite a successful Kasai procedure. A liver transplant operation removes the damaged liver and replaces it with a new liver from a donor. MCT (medium-chain triglyceride) oil or infant formulas with MCT (Portagen or Pregestimil) may be recommended to add extra calories to help your child grow.
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