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Doctors Can't Determine Cause Of New Liver Disease

POSTED: 4:09 pm EST February 25, 2008
UPDATED: 12:42 pm EST February 26, 2008

The following is a transcript of a report by medical editor Marilyn Brooks that first aired Feb. 25, 2008, on WTAE Channel 4 Action News at 5 p.m.


A mysterious liver disease is now under the microscopes of researchers nationwide, including those at Children's Hospital in Pittsburgh.

The disease can be fatal or lead to a liver transplant, but the cause is unknown, and scientists want to change that.

Primary sclerosing cholangitis is relatively uncommon. The symptoms are so vague that patients sometimes go for months or years before they learn they have a potentially fatal disease.

Ten centers nationwide are joining together to find out all they can on the disease.

Joey Harim was diagnosed with the disease, and has had many tests done to figure out what causes it.

Sclerosing cholangitis causes inflammation and scarring of the liver bile ducts, which could destroy the liver.

Harim began vomiting, and got a fever and a cough in 2006. Unable to find a cause, his pediatrician sent him to Children's Hospital in March 2007.

"The emergency room doctor ran a lot of blood work, and he found it," said Harim's mother, Cherrie Harim. "I forget his name. He found that the liver enzymes were really bad."

Harim's was caught early. He's on two medicines and should get better.

Sclerosing cholangitis affects one in every 100,000 people. The real mystery of it is that there is no known cause, which means there is no perfect fix.

Doctors in 10 centers will collect information on children with PSC to try to get results.

"What happens to kids, how they present with the illness, what happens to their illness over time, how they respond to treatments," said director of hepatology at Children's Hospital, Dr. Benjamin Shneider.

Primary sclerosing cholangitis progresses slowly, so it can be years before symptoms develop including. Those symptoms include fatigue, jaundice, chills and fever. Children usually have autoimmune hepatitis as well. The body's immune system attacks the liver cells and it's not infectious.

Doctors hope that in five years, they'll know how to save livers and lives from the disease.


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