Liver Disease | Biliary Atresia

Biliary Atresia

What is Biliary Atresia?

Biliary Atresia is a rare disease of the liver and bile ducts that occurs in infants. Infants with biliary atresia appear to be normal at birth. Symptoms of the disease appear or develop about two to eight weeks after birth.

When a baby has biliary atresia, bile flow from the liver to the gallbladder is blocked. This causes the bile to be trapped inside the liver, quickly causing damage and scarring of the liver cells (cirrhosis), and finally liver failure. Bile is a digestive liquid that is made in the liver. It travels through the bile ducts to the small intestine, where it helps digest fats.

Why Does Biliary Atresia Occur?

The cause of biliary atresia is not known. The cause of biliary atresia is not known. Suggested causes include viral infections or an over-response of the body's own immune system. It is not a hereditary disease and is unlikely to occur more than once in a family. Biliary atresia only occurs about once in every 15,000-20,000 births worldwide.

Symptoms Of Biliary Atresia

Symptoms of Biliary Atresia usually occur between 2 and 8 weeks of life. The first signs may include:

Jaundice - A yellow appearance of the skin and whites of the eyes (sclera). The jaundice is due to the build-up of excess bilirubin throughout the body as the liver isn't able to clear it properly.

Urine - Appears very dark yellow or brown: this is due to the increased bilirubin in the bloodstream, which then passes to the kidneys.

Stools - Appear pale or clay-coloured. This happens because there is little or no bile reaching the intestine to colour the bowel movements.

Enlarged Liver - Feels larger and harder than normal.

Poor Weight Gain - Infants with biliary atresia often do not gain weight. Bile is required to digest and absorb most types of fat.

How to Spot Signs of Biliary Atresia

Usually the first signs that a parent should be vigilent about are the palse stools and jaundice. The jaundice can sometimes be misinterpreted as the jaundice usually seen with first borns and thus considered normal. In Biliary Atresia, this jaundice does not improve within 1 to 2 weeks.

The sooner Biliary Atresia is identified, the sooner it can be treated ( usually with the Kasai Procedure) and therefore minimise the long term damaging effect on the liver.

How to Prevent The Affects of Biliary Atresia

Instructions

Step 1:
Know how the condition affects your infant's body. This progressive inflammatory process starts soon after birth and first affects the delicate outer ducts of the liver. White blood cells flood the ducts, damaging and possibly dissolving them. The bile backs up in the liver and if left untreated will require a liver transplant.
Step 2:
Understand that the cause is still unknown. Biliary atresia is not hereditary, is not contagious and is not caused by any activity on the mother's part. A recent study suggests that a viral infection could cause it in susceptible infants. Ten percent of children diagnosed with the condition also have a congenital defect of the heart, intestine, spleen or blood vessels.
Step 3:
Recognize the symptoms. The child will appear normal at birth but start to look jaundiced after a few weeks due to the build up of bile. Before long the child will act irritable, experience weight loss and have a swollen abdomen from the enlarging liver.
Step 4:
Have a doctor confirm the diagnosis. She will administer a blood test to rule out other possible causes of jaundice or liver obstruction and then conclude with surgical exploration to confirm the damage to the liver ducts.
Step 5:
Submit to the recommended treatment for biliary atresia, known as the Kasai procedure, which removes the ducts and then attaches the small intestine directly to the liver. The surgery successfully reestablishes bile flow in 80 percent of infants under three months old, but if it fails then the next step is a liver transplant.
Step 6:
Prepare for possible complications from the Kasai procedure. Administer any special dietary vitamins and fats prescribed for the infant to assist with proper absorption of vitamins and minerals and stimulate normal growth and development. Prepare for complications from the treatment. Get regular check-ups to watch for bacteria build up and scar tissue in the small intestine, both of which may need a liver transplant if they become serious.

Tips & Warnings

Early identification of the condition is critical because the Kasai procedure has a low success rate in infants older than three months.

Source

Source:

Cincinnati Childrens Hospital Medical Center http://www.cincinnatichildrens.org/

The American Liver Foundation http://www.liverfoundation.org/

The Childrens Hospital Westmead http://www.chw.edu.au/parents/factsheets/biliary_atresia.htm

What is the outcome for children with Biliary Atresia?

If left untreated, the result of blocked bile flow is damage to the liver such that few children survive beyond the age of two. When bile flow is only partly restored by surgery, the complications of cirrhosis will gradually develop.

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