Children's Hospital 1 of 10 pediatric hospitals in US selected to study liver disease in kids

Children's Hospital of Pittsburgh of UPMC has been selected to join an effort among select centers in the United States and Canada to collect and study information necessary to understand the possible causes and treatment of a destructive liver disease called primary sclerosing cholangitis (PSC).

The Studies of Primary Sclerosing Cholangitis, or STOPSC, is a multi-center research effort initiated, funded and organized by The Musette and Allen Morgan Jr. Foundation. PSC is a destructive liver disease of unknown origin that occurs in both males and females and in children and adults. About one person in every 100,000 will get the disease, and currently there is no proven medical treatment for PSC.

Researchers across the country will establish a database and specimen storehouse in an effort to make the information available to other qualified researchers so future studies can be conducted. The major goal of this study is to help develop better ways to detect the disease, find out how effective current treatments are, and conduct more research studies in the future.

“This effort is so important for many reasons – most important is to better understand the disease and how to treat it. By combining all of our information and research, together, we can tackle this devastating disease head on,” said Benjamin L. Shneider, MD, director of the Pediatric Hepatology Program at Children's Hospital, who is a member of the American Association for the Study of Liver Diseases. “At Children's Hospital of Pittsburgh of UPMC, we are dedicated to helping children with liver disease by exploring all treatment options — maximizing liver function, minimizing complications and maintaining growth and development — all to improve the quality of life for each child and family.”

The study also will include adults and children with a diagnosis of PSC/autoimmune hepatitis (AIH) overlap syndrome and children diagnosed with AIH, because the clinical aspects and the possible causes of these diseases may be related. AIH is usually quite serious and, if not treated, gets worse over time. It's usually chronic, meaning it can last for years, and can lead to cirrhosis (scarring and hardening) of the liver and eventually liver failure. In addition, many individuals with PSC will also get inflammatory bowel disease.

Children's is one of only 10 pediatric hospitals selected to participate in this national study. Other hospitals include the Mayo Clinic, Children's Hospital of Denver, Children's Hospital Boston, and Mount Sinai Medical Center, to name a few.

http://www.chp.edu/