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Biliary atresia.

What is biliary atresia?

Biliary atresia is a serious disease of the very young infant. It results in inflammation and obstruction of the ducts which carry bile from the liver into the intestine. When bile cannot flow normally, it backs up in the liver (a situation called biliary "stasis"). This causes "jaundice," or a yellowing of the skin, and cirrhosis. Cirrhosis occurs when healthy liver cells are destroyed, in this case by disease, and replaced with scar tissue. This scarring interferes with blood flow through the liver, causing more cell damage and scarring.

What are the first signs of biliary atresia?

The symptoms of biliary atresia are usually evident between two and six weeks after birth. The baby will appear jaundiced, and may develop a large, hardened liver and a swollen abdomen. The stools are usually pale and the color of clay; the urine appears dark.

Some babies may develop intense itching, or "pruritus" which makes them extremely uncomfortable and irritable. The exact cause of this itching is not yet known, although researchers have found a connection between it and the backup of bile.

What causes biliary atresia?

The cause of biliary atresia has not yet been discovered, but researchers suspect that a viral infection around the time of birth may cause the fetal bile docts to become inflamed and obstructed.

The disease affects approximately one infant in every 20,000 live births. Girls are affected slightly more othen than boys, but no racial or ethnic group appears to be more affected than any other.

Biliary atresia is not a hereditary condition (although in some very rare cases, more than one infant in a family may be affected). Many parents experience feelings of guilt, but they should be reassured that nothing they have done caused their child's illness.

How is biliary atresia diagnosed?

There are many liver diseases which cause symptoms similar to those of biliary atresia. Consequently, many tests may have to be performed before biliary atresia can be diagnosed conclusively.

Every effort should be made to search for any of the causes of jaundice which might be confused with biliary atresia. This involves blood and urine tests; liver function tests; blood counts and a test for clotting function. A painless examination using ultrasound (ECHO) is often done to study the liver and determine the size of the bile ducts and gall bladder.

Other tests which are often used are specialized X-ray techniques or radioactive scans of the liver which can be helpful in focusing on the true abnormality. A liver biopsy, in which a tiny sample of the liver is removed with a needle, allows the physician to examine the liver tissue microscopically.

What about treatment?

The most successful treatment for biliary atresia to date is a type of surgery which creates drainage of bile from the liver when the ducts have become completely obstructed. This operation is called the Kasai procedure (hepatoportoenterostomy) after Dr. Morio Kasai, the Japanese surgeon who developed it.

In the Kasai procedure, the surgeon removes the damaged ducts outside of the liver (extrahepatic) and replaces them with a length of the baby's own intestine, which acts as a new duct. Sometimes this new duct is brought onto the baby's abdomen, sometimes not.

The aim of the Kasai procedure is to allow excretion of bile from the liver into the intestine via the new duct. The operation accomplishes this about 50% of the time. In those who respond well, bile begins to flow several days after surgery, and jaundice usually disappears after several weeks.

In the remaining 50% of cases where the Kasai procedure does not work, the problem usually lies in the fact that the obstructed bile ducts are "intrahepatic" or inside the liver, as well as outside. No surgical procedure has yet been developed to correct these interior ducts.

What happens after surgery?

The aim of treatment after surgery is to encourage normal growth and development. If bile flow is good, the child is given a regular diet. If bile flow is reduced, a low fat diet is recommended as bile is required to aid in the absorption of fats and vitamins: multiple vitamins, vitamin B complex, and vitamins E, D, and K can be given as supplements.

Is the Kasai procedure a cure for biliary atresia?

Unfortunately, despite bile flow, the Kasai procedure is not a cure for biliary atresia. It can only be used on those infants whose extrahepatic bile ducts are obstructed. And for reasons which are still unknown, liver damage often continues, and, eventually, cirrhosis and its complications appear.

What are the complications?

Patients with cirrhosis have changes in blood flow through the liver which may produce abnormalities such as easy bruising of the skin, nosebleeds, retention of body fluid, and enlarged veins called varices in the stomach and esophagus. Increases in pressure in these veins can make them leaky, and internal bleeding results. This can usually be stopped. In some cases, an operation (called a shunt operation) to lower the pressure may be required.

As the disease progresses, other complications may occur. While all infants tend to be sleepy after eating, those with biliary atresia may experience excessive sleepiness after eating protein, due to increased nitrogen products in the bloodstream. The child may also suffer from an increased risk of infection.

What can be done about these complications?

Following the Kasai operation, infection in the bile ducts (cholangitis) is common. This is usually treated in the hospital using intravenous antibiotics and may be continued at home with oral antibiotics.

If retention of body fluid occurs, it can be treated with diuretics and potassium replacement.

Jaundice or itching can often be treated successfully with two medications - phenobarbital and cholestyramine.

What is the outlook for a baby with biliary atresia?

The extent and type of liver damage differ in each baby with biliary atresia. Some infants respond to surgery; others do not. If bile continues to flow, long term survival is possible. However, it is presently impossible for a physician to determine in advance which baby is likely to respond to treatment.

Is liver transplantation the solution?

Liver transplantation is an option which is becoming increasingly available to victims of certain liver diseases. The survival rates for transplant recipients are increasing dramatically with improved surgical techniques and the development of new drugs which help overcome the problem of organ rejection.

In children with biliary atresia, liver transplantation is generally not attempted until the Kasai procedure has been performed. If this operation is not successful, and if complications of the resulting cirrhosis become severe and life threatening, liver transplantation may be attempted. It has been successful in numerous cases. However, as in all organ transplantation, success depends greatly upon the timely availability of suitably matched organs for donation, the time factor involved and other factors which are only now being investigated.

What can the family do?

Watching a young infant suffer from biliary atresia is a devastating experience. It can also be frustrating, because so little is known about the disease. Feelings of anger and helplessness are not uncommon. What does one do when there is so little one can do?

Many parents have found it helpful to learn as much as they can about the disease. Talk to your physician, inquire about specialists, request any literature on the subject.

Perhaps the biggest comfort for parents is to discuss the problem with others who have or are currently going through a similar tragedy. Finding out that they are not alone, that others feel the way they do, and learning how other parents are coping with their child's disease, is often a great comfort.

Where can parents turn for support?

The American Liver Foundation recognizes that parents of children with biliary atresia need help in coping with the immense strain of this chronic illness. To meet this need, ALF is continually organizing and coordinating mutual help groups through its chapters to provide emotional support for families, make referrals to specialists where appropriate, and to keep people aware of the latest research developments.

Will there ever be a cure for biliary atresia?

There can be no cure for biliary atresia until the cause of the disease can be determined. Researchers are focusing on trying to find this cause, but a great deal of work still needs to be done. More research into how the liver works is also vital.

Research is the key that will unlock this mystery. ALF, a national voluntary health agency, is dedicated to providing the means for more research, and to helping people understand more about the liver and liver disease.
COPYRIGHT 1991 American Liver Foundation
No portion of this article can be reproduced without the express written permission from the copyright holder.
Copyright 1991 Gale, Cengage Learning. All rights reserved.

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Publication:Pamphlet by: American Liver Foundation
Article Type:pamphlet
Date:Sep 23, 1991
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