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Biliary Atresia is a liver disease that affects the bile ducts of the newborns. This disorder is very rare but it may cause fatal complications in the affected children. This article discusses biliary atresia in detail including treatment options.

Biliary atresia is a rare liver disease affecting newborn infants. Its occurrence is relatively rare (one in every 10,000 children) and the causes remain unknown. Current treatment strategies are only moderately successful at curing the affected child.

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What Is Biliary Atresia?

Biliary means "related to bile" whereas "atresia" is a Greek word that means "absence". Biliary atresia is a disease caused by damage to the bile ducts. Bile ducts drain bile, produced by the liver, into the gallbladder and small intestine. Bile helps in emulsifying fats and in cholesterol reabsorption. Damage results in the loss (atresia) of these ducts, causing bile to accumulate in the liver. This build-up may also damage the liver, causing scarring of liver tissue – cirrhosis of the liver. This condition may be irreversible and worsen over the time. After one to two years, the liver may cease to function, resulting in severe liver cirrhosis and the acute need of a liver transplant.

Causes Of Biliary Atresia

Biliary atresia is a rare disease, and affects more newborn girls than boys. It more commonly occurs among Asian and African-American populations as compared to Caucasians. Within a single family, it may affect only one child, or only one in a pair of twins.

No definitive cause of biliary atresia has been discovered.

The disease can occur because the bile ducts were malformed during pregnancy, or they were damaged by the baby’s immune system because of a viral infection that occurred after birth.

Scientists have, however concluded that biliary atresia is:

  • Not hereditary in nature
  • Not contagious
  • Not preventable
  • Not caused by any medication taken during pregnancy

Complications Of Biliary Atresia

Biliary atresia itself may not cause severe harm to the patient. However, after the development of complications, it may become difficult for a child to survive. About 10 to 15 percent of infants diagnosed with biliary atresia are found to have the following complications:

  • Heart complications (abnormalities in the inferior vena cava)
  • Pre-duodenal portal vein
  • Polysplenia (many small accessory spleens)
  • Intestinal abnormalities (malrotation, situs inversus)

Biliary Atresia - Common signs and Symptoms

Babies begin exhibiting symptoms of biliary atresia within the first two weeks to two months of birth. Common symptoms are as follows: 

  • Jaundice

The affected infant appears normal at birth, but develops jaundice within two to three weeks. Jaundice is characterized by yellowing of the skin and the whites of the eyes. It occurs due to the build-up of bilirubin (pigment in bile) in blood. If the jaundice becomes severe, weight loss and irritability may develop as well.

  • Alcoholic/Clay-colored Stools

This occurs because bile cannot flow out of the liver into the small intestine, thus unable to remove acidic metabolic compounds from the body. The abdomen may become firm and swollen as well.

  • Dark Urine

This also occurs due to the build-up of bilirubin in the blood. The copious amounts filtered by the kidneys and excreted in the urine cause the color of the urine to change.

Other symptoms include:

  • Enlarged spleen
  • Foul-smelling and floating stools
  • Retarded growth
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