What is Biliary Atresia?

A serious and rare liver disease that affects newborns, biliary atresia happens in one in 10,000 babies. It is more likely to occur in females and Asian or Africa-American children. Causes are not known and treatments are not fully affective.

Symptoms of Biliary Atresia

The signs of biliary atresia start with jaundice, or a yellowing of the skin and the eye whites. When the liver doesn’t rid the blood of bilirubin, this can occur. Other symptoms are a dark urine and lighter color in the bowel movements. The most reliable sign of it is newborns having white or pale gray bowel movements after the age of two weeks.

Liver Damage

Liver damage from biliary atresia is from bile duct injury and loss. The loss of bile ducts lets the bile stay in the liver and not pass into the intestines to digest food. This build up causes scarring and loss of tissue in the liver, leading to cirrhosis and liver failure. This can occur as early as the first two years of life.

Diagnosis

Diagnosis of this condition is from symptoms, blood tests, x-rays, and possibly a liver biopsy. There will be a bilirubin test to check for the amounts in the blood. There may be ultrasounds of the liver or the abdomen to see if the liver is enlarged, or if there are tumors or cysts. There can be a liver scan to look at the bile ducts and liver. Lastly, a liver biopsy can take a small piece of tissue and test it, helping to rule out other liver conditions like hepatitis.

Treatment of Biliary Atresia

The treatment options for this condition include surgery, the Kasai procedure, and a liver transplant. Surgery is done to have exploratory surgery on the liver and bile ducts. If there is biliary atresia a Kasai procedure is done. The Kasai procedure removes the bile ducts and loops the intestine to replace it. It is also called a hepato-portoenterostomy. If this procedure fails to work there can be a liver transplant.

This is a rare condition that has devastating effects on the babies that it affects. Catching it early, before too much liver damage has been done, is the best way to treat it. If a baby is still in jaundice after one or two weeks of life, have it checked for this condition. Babies can be naturally jaundice, but it usually clears itself in a week or two, not clearing is a very real sign of liver damage.

Source: The National Digestive Diseases Information Clearinghouse (NDDIC)


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