They will have a scar from their initial surgery, but otherwise look and act normal.We now care for a group of young adults who were the first generation of patients who underwent a Kasai operation. Researchers have reported varying results over the past three decades. It is the most common cause of liver transplants in children in the U.S.

To help your child grow, we often use a special type of formula that has easy-to-absorb fat and we also prescribe special vitamins.Your child may also be prescribed other medicines such as antibiotics to prevent infection of the bile ducts and ursodeoxycholic acid to promote bile flow.Although the Kasai procedure provides bile drainage for many children, it does not usually cure the child of ongoing inflammation in the liver and bile ducts.Your child will need close follow-up by a specialist for life. Protein deficiencies may occur due to liver damage. This is less common. It is thought that approximately 80 percent of patients with biliary atresia will require liver transplantation by the age of 20. When the Kasai procedure is done at an early age (younger than three months), about 80 percent of patients have some bile flow. Most often, symptoms develop within the first two weeks to two months of life. Your doctor will consider the possibility of transplantation if there are complications that are not responding to other medical approaches.Typical reasons to consider liver transplantation include:Your doctor will refer you to the liver transplant team before these problems are life threatening. Doctors treat biliary atresia with a surgery called the Kasai procedure and eventually, in most cases, a liver transplant. Nutritional guidelines may include the following: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. It is reddish-brown, weighs about 1½ to 2 pounds in a child (depending on age), and holds about 13 percent of the body's blood supply at any given moment. Median age at Kasai operation was 60 days and was stable over the study period. When the spleen is large, your physician may prescribe a spleen guard to be worn during gym or during certain activities to protect the spleen from injury.Approximately half of all children born with biliary atresia will need a liver transplant by the time they are 2 years old. The operation connects the bile draining from the liver directly to the gastrointestinal (GI) tract. When blocked ducts prevent bile from being excreted, the liver becomes damaged.The cause of biliary atresia is a mystery. If that happens, there is an increase in the blood flow through certain blood vessels. The body needs bile to aid digestion and carry wastes from the liver out of the body. Fetal biliary atresia. About one in 8,000 to 20,000 get this rare disorder.Trying to figure out what causes this problem or how we can improve life with biliary atresia is one of the highest priorities of the physicians caring for children with biliary atresia at The Children’s Hospital of Philadelphia.As far as we know, you did not cause your child to get biliary atresia because of anything you did during pregnancy. It appears after birth, most often when a baby is about 2 to 4 weeks old. But these statistics may change as we learn more about biliary atresia and modify our treatment approaches.Those living with their native liver and reaching adulthood, can expect to go to school, have jobs and even families, if they desire. Bile byproducts enter the intestine and leave the body in the feces; the kidneys filter out the blood byproducts, which leave the body in urine.The liver carries out many other important jobs, including:Infants with biliary atresia usually appear healthy at birth. When that happens, your child may vomit a large amount of blood. This delays (or in about 25% of children, eliminates) the need for a liver transplant. Vitamin deficiencies may also occur. The hepatic duct transports bile produced by the liver cells to the gallbladder and duodenum (the first part of the small intestine).One of the liver's primary functions is to filter the blood of toxins and other harmful substances, which are then excreted into bile or blood. 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.In general, the long-term outlook for patients with biliary atresia is excellent. Jaundice develops in the first few weeks of life.The less-common embryonic or fetal form occurs in 10-35 percent of biliary atresia cases.

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