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BILIARY ATRESIA

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BILIARY ATRESIA

Biliary atresia is a rare and chronic disease of the liver and bile ducts that become evident shortly after birth

  • The bile ducts are tubes in the liver that normally allow bile, a liquid produced by the liver cells, to drain into the intestines and kidneys where it helps digest fats
  • This network of channels and duct is referred to as the biliary system.
  • When the biliary system is working properly, it allows bile to drain from the liver into the intestines and kidneys
  • When a baby has biliary atresia, bile ducts in the liver are absent, narrowed, or blocked and prevents the draining of the bile from the liver
  • This allows the bile to be trapped inside the liver where it damages liver cells rapidly, causing scarring (cirrhosis), and eventually liver failure
  • Biliary atresia occurs in 1 out of 10,000 live births and is more prevalent in girls than boys
  • It is a life-threatening condition and is fatal if left untreated.
  • In addition to causing liver damage, biliary atresia also affects numerous processes that allow the body to function normally

CAUSES

The exact cause of the condition is not known

Some experts believe that the problem with the bile ducts may occur while the liver was developing during pregnancy. Other believe that auto-immune mechanism may be responsible; the bile ducts may be damaged by the body’s immune system in response to a viral infection or toxic substance after birth.

What is known for sure is that biliary atresia only affects newborns, it is not contagious, it is not caused by anything the mother did during pregnancy, and it is not hereditary.

SYMPTOMS

Symptoms of biliary atresia appear about two to eight weeks after birth, and may include:

  • Dark urine
  • Jaundice
  • Weight loss and irritability
  • Clay-colored stools (acholic stools)
  • Distended abdomen

DIAGNOSIS AND TREATMENT

To get a correct diagnosis, the doctor may need to carry out the following diagnostic procedure:

  • Blood tests to check for liver function abnormalities
  • Imaging tests such as X-rays to look for enlarged liver and spleen or an abdominal ultrasound
  • A liver biopsy
  • Diagnostic surgery to confirm if an infant has the condition
TREATMENT

There are no medications that can unblock the bile ducts or encourage the growth of new bile ducts where none were present before. Treatment options for biliary atresia include:

A Kasai procedure where the damaged ducts outside of the liver are removed and the bile flow from the liver to the intestine is re-established by joining the two directly. This procedure is not curative but it will allow a child with biliary atresia live longer and have a good quality of life

Liver transplantation, where the damaged liver is removed and replaced with a new liver from a donor, may be carried out. A liver transplantation is the only cure for biliary atresia.

Children with biliary atresia may need more calories so the doctor may recommend nutritional guidelines including:

  • Providing the child with a good, well-balanced diet
  • Supplementing diet with vitamins
  • Providing the child with high-calorie liquid feedings if the child is too sick to eat normally