Q&A: Understanding Pediatric Liver Disease

Biliary atresia only affects infants. It’s important to understand that bile is a digestive liquid made in the liver. It travels through the bile ducts to the small intestine, where it helps digest fats. In biliary atresia, the bile ducts become inflamed and blocked soon after birth. Bile remains in the liver, where it starts to destroy liver cells rapidly and cause cirrhosis, or scarring of the liver.

Over all, it’s found in one in 10,000 births in the United States—about 300 every year. About one in 10 babies with biliary atresia have other congenital defects.

It’s unclear. In some infants, the condition is present from birth. An early viral infection may be linked to biliary atresia. As it isn’t hereditary, parents shouldn’t blame themselves. It isn’t caused by anything an expectant mother did or didn’t do.

No

Between two and six weeks after birth, the baby appears jaundiced, with yellowing of the skin and whites of the eyes. The liver may harden and the abdomen could become swollen. Stools appear pale grey and the urine may appear dark. Some babies may develop intense itching.

With a surgical procedure in which the blocked bile ducts outside the liver are replaced with a length of the baby’s own intestine, which acts as a new duct. This surgery is called the Kasai procedure after Dr. Morio Kasai, the Japanese surgeon who developed it. It drains bile from the liver into the intestine through the new duct.

Without successful treatment, few children with biliary atresia live beyond age two. The only long-term solution may be a liver transplant.

Sadly, there’s a shortage of organ donors across America. In 2012, there were 410 liver transplants in the U.S. involving children under the age of 10. At present, a total of 326 children under the age of 10 are listed for liver transplants—and that includes 47 babies under the age of one.

An estimated 23,000 to 46,000 children in the United States are infected with chronic Hepatitis C or HCV for short, which results in an inflamed liver.

Babies born to a mother with HCV have the highest risk. According to Michael R. Narkewitz MD (4), if a mother has HCV, her child has a one in 20 chance of becoming infected at birth. Adding to the problem, it’s difficult to detect because you can have HCV for 15 years or longer before being diagnosed. Unlike hepatitis A and B, there’s no vaccine to prevent it.

Dr. Narkewitz says for children infected by their mother, “up to 40 percent will clear the virus on their own without treatment by two years of age. There are reports of children clearing the virus on their own as late as seven years of age.” All the same, he recommends that children age three and over with aggressive liver disease should be treated with a combination of interferon and ribavirin.