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Diseases

Biliary Atresia

Biliary atresia is a rare disease of the liver and bile ducts that occurs in infants. About one in 15,000 to 20,000 babies will have defective bile ducts. Bile ducts are tubes that drain the bile, a liquid produced by the liver, from the liver to the intestines. Bile is important to digest fats and it also contains products that have to be excreted out of the liver. This network of channels and ducts is called the biliary system. When the biliary system is working normally, the bile drains from the liver into the intestines.

  • Occurs when the bile ducts do not form during pregnancy
  • Bile ducts may be can get damaged by the body’s immune system in response to a viral infection acquired after birth

Risk Factors

  • Affects girls more than boys
  • Asians and African-Americans are affected more frequently than Caucasians
  • It is not an inherited condition so in a single family only one child may get affected
  • No link to medications taken during pregnancy has been established
  • About 15% of infants may be born with defects in the heart, spleen, veins of the liver, or intestines

 

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    Symptoms

    Babies with biliary atresia usually appear healthy when they are born; symptoms of the disease appear or develop about two to eight weeks after birth. They include:

    • Yellowish coloration of the skin and whites of eyes (jaundice); biliary atresia is one of the common causes of jaundice in new-born infants. Normally an immature liver will result in jaundice that usually disappears in the first week to 10 days of life.
    • Dark urine that can stain the diaper or nappy yellow is caused by the build-up of bilirubin in the blood. The bilirubin is then filtered by the kidney and removed in the urine.
    • Characteristic white colored stools result from the inability of the bile or bilirubin coloring being emptied into the intestine
    • Swollen abdomen may be from a firm, enlarged liver
    • Weight loss and irritabilityusually develop when the level of jaundice increases

    Occasionally bleeding from the nose or umbilical cord may be present

    Diagnosis

    Investigations include:

    Routine blood tests including liver function tests to assess liver dysfunction

    • Liver tests is usually a combination of 3 tests which confirm the diagnosis:
      • Increase in the blood level of direct bilirubin
      • Minor increase in liver enzymes serum glutamic-oxaloacetic transaminase (SGOT) and serum glutamic pyruvic transaminase (SGOT)
      • Marked raise in the liver enzyme which is markedly raised is gamma-glutamyl transpeptidase (GGTP).
      • They may also identify the cause of jaundice

    Imaging studies

    Abominal ultrasound

    • Can reveal presence or absence of gall bladder, liver size
    • Common bile duct is not seen

    HIDA scan

    Is a special type of imaging test by injecting a dye. A normal liver will take up the dye and excrete into the intestine.

    • In biliary atresia, the liver uptake is usually normal but the dye does not pass into the intestine. Sometimes a 5 days of syrup phenobarbitone to improve the test efficacy is given before undergoing this procedure
    • Many other conditions also will show the dye not passing in to the intestine
    • If the HIDA scan is normal (showing dye excreting to intestine), it can be confirmed that biliary atresia is not present

    Treatment

    Biliary atresia cannot be treated with medication; surgical procedures are the only option. Biliary atresia is a surgical emergency in a newborn

    • A surgical procedure called the Kasai procedure is undertaken if biliary atresia is confirmed with the diagnostic cholangiogram as a second step of surgical treatment. The procedure is named after the Japanese surgeon who invented it.
      • The procedure re-establishes the bile flow from the liver into the intestine by performing a hepatoportoenterostomy, by which the liver surgeon removes the damaged ducts outside of the liver and identifies smaller ducts that are still open and draining bile.  The surgeon then attaches a loop of intestine to this portion of the liver, so that bile can flow directly from the remaining healthy bile ducts into the intestine.
    • Best results are seen if the surgery is performed within 60 days of birth and is successful in 60 to 85% of the patients. If an infant is 4 months old, Kasai procedure is unlikely to be helpful
    • Allows babies to grow and have fairly good health for atleast a few years

    In 15-40% of patients the Kasai procedure does not work, then only a liver transplantation can correct this problem. The reasons for this include:

    • Extensive of liver damage (cirrhosis) at the time of surgery
    • Progressive cirrhosis and the development of portal hypertension (high blood pressure in the portal vein that carries blood to the liver)
    • The number and size of microscopic ducts in the scarred tissue that can drain bile

     Liver transplant

     Liver transplantation is the only curative option for this dreadful condition. It removes the damaged liver and replaces it with a new liver from a donor. After undergoing a successful Kasai procedure, many symptoms begin to reappear and get worse and liver transplant then is the only option

    • 50% infants who have had a Kasai procedure require liver transplantation before age 5
    • 85% of all children who have biliary atresia will need to have a liver transplant before they are 20 years old. The remaining 15% will have some degree of liver disease, which can be managed without having a transplant
    • After a transplant, lifelong care and follow up with the transplant team is required

    Role of nutrition in biliary atresia

    • Due to poor absorption of fat by the intestine, the caloric requirement is 1.5 times the normal requirement. To increase the calorie intake, adding medium-chain triglyceride (MCT) oil to foods and liquids or infant formulas is recommended
    • Due to liver damage, there may also be a loss of protein and vitamins
      • The protein intake should be 3-4 gm/kg/day
    • Fat soluble vitamins like A, D E and K need to be supplemented
      • B complex vitamins need to be given 2-3 times the recommended daily allowance