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Biliary atresia is a chronic, progressive liver problem that becomes evident shortly after birth. Tubes inside and outside the liver, called bile ducts, normally allow a liquid produced by the liver called bile to drain into the intestines and kidneys. Bile aids in digestion and carries waste products from the liver to the intestine and kidneys for excretion. In biliary atresia, bile ducts that are located inside or outside the liver are blocked. When the bile is unable to leave the liver through the bile ducts, the liver becomes damaged and many vital body functions are affected.
The cause of biliary atresia is not known. Some researchers and physicians believe that babies are born with biliary atresia, implying the problem with the bile ducts occurred during pregnancy while the liver was developing. Others believe that the disease begins after birth, and may be caused by exposure to infections or exposures to toxic substances.
Biliary atresia does not seem to be linked to medications the mother took, illnesses the mother had, or anything else the mother did during her pregnancy. Currently, there is not a genetic link known for biliary atresia. The disease is unlikely to occur more than once in a family.
Biliary atresia causes liver damage and affects numerous important processes that allow the body to function normally. It is a life-threatening disease and is fatal without treatment.
Infants with biliary atresia usually appear healthy at birth. Most often, symptoms develop between two weeks to two months of life, and may include:
Jaundice is a yellow discoloration of the skin and whites of the eyes due to an abnormally high level of bilirubin (bile pigment) in the bloodstream, which is then excreted through the kidneys. High levels of bilirubin may be attributed to inflammation or other abnormalities of the liver cells, or blockage of the bile ducts. Jaundice is usually the first sign, and sometimes the only sign, of liver disease.
Symptoms of biliary atresia may resemble other liver conditions or medical problems. Please consult your child's physician for a diagnosis.
A physician or healthcare provider will examine your child and obtain a medical history. Several diagnostic procedures are done to help evaluate the problem and may include the following:
The test that gives the most definitive diagnosis is a liver biopsy. A tissue sample is taken from your child's liver and examined for abnormalities, allowing biliary atresia to be distinguished from other liver problems.
Specific treatment for biliary atresia will be determined by your child's physician based on the following:
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. Until that happens, biliary atresia will not be curable. However, two different operations can be done that will allow the child with biliary atresia to live longer and have a better quality of life. Your child's physician can help determine whether either of these operations are an option.
After surgery, the new liver begins functioning and the child's health often improves quickly. After a liver transplant, children will need to take medications to prevent the body from rejecting the new organ. Rejection occurs due to one of the body's normal protective mechanisms that helps fight against invasion of viruses, tumors, and other foreign substances. Anti-rejection medications are taken in order to prevent this normal response of the body from fighting against the transplanted organ. Frequent contact with the physicians and other members of the transplant team is crucial after a liver transplant.
Before your child has either one of these operations, nutrition may be a problem. With biliary atresia, not enough bile reaches the intestine to assist with the digestion of fats in the diet. Protein deficiencies may occur due to liver damage. Vitamin deficiencies may also occur. Children with liver disease require more calories than a normal child because of a faster metabolism.
Your physician may recommend that a pediatric nutritionist make recommendations regarding your child's diet. Nutritional guidelines may include the following:
After surgery, your child's digestion may return to normal, or you may still need to give extra vitamins and/or work with your child's diet. Please consult your child's physician for recommendations.
Many factors affect the long-term outlook for these children. Some of them include:
As many as 80 percent of children who have the Kasai portoenterostomy will eventually require a liver transplant. After liver transplant, the child's health will usually improve; however, a rigorous medical regimen must be followed.
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