University Joins Network to Study Rare Liver Diseases in Children

University Joins Network to Study Rare Liver Diseases in Children

Sep 29, 2014 4:32 PM

(SALT LAKE CITY)—The University of Utah School of Medicine and Primary Children’s Hospital have been awarded a $1.6 million grant to become the newest member of ChiLDReN, a 13-center National Institutes of Health (NIH) network of academic pediatric centers dedicated to researching rare childhood liver diseases.

The Intermountain West Clinical Center for ChiLDReN is based at the U of U and Primary Children’s Hospital (PCH). Stephen L. Guthery, M.D., U of U professor of pediatrics, is the principal investigator.

The mission of ChiLDReN (the Childhood Liver Disease Research Network) is to improve the care of children with liver diseases by better understanding what causes them, improving how to diagnose them and developing and testing treatments for them.

The network studies seven liver diseases, including: biliary atresia, an inflammatory condition of the bile ducts that occurs shortly after birth and is the leading indication for childhood liver transplantation; progressive familial intrahepatic cholestasis syndromes, which are genetic conditions resulting in abnormal bile processing in the liver cell; and Alagille syndrome, a multi-system genetic disorder that can effect the liver.

In addition to these diseases, Guthery, his co-investigators and the other centers also will research alpha-1-antitrypsin deficiency, an inherited condition that causes the liver to make and store large amounts of an abnormal protein; bile acid synthesis and metabolism defects, in which the body does not make bile acids correctly and the intestine cannot absorb and use fats correctly; idiopathic neonatal hepatitis, a condition of unknown cause in which the liver becomes inflamed shortly after birth; mitochondrial hepatopathies, a group of liver diseases in which mitochondria – often referred to as the “cells energy plants” – function improperly.

As part of ChiLDReN the University and PCH join some of the most prestigious pediatric liver disease centers in the United States and Canada, according to Guthery. “This is a great opportunity to work with other top pediatric liver centers to make a difference in the lives of these young patients,” Guthery says. “I am optimistic that through ChiLDReN we will make discoveries that will improve the understanding and treatment of rare liver diseases.”

The ChiLDReN network includes academic pediatric centers such as Cincinnati Children’s Hospital, Children’s Hospital of Philadelphia, University of California, San Francisco, The Hospital for Sick Children, Toronto, and the Baylor (Texas) College of Medicine.

Funding for the Intermountain West Clinical Center for Children comes through the National Institute of Diabetes and Digestive and Kidney Diseases, which is part of the NIH.

Guthery’s co-investigators include Linda Book, M.D., professor of pediatrics (gastroenterology and hepatology); M. Kyle Jensen, M.D., assistant professor of pediatrics (gastroenterology and hepatology); Rebecka Meyers, M.D., professor of surgery (pediatrics); Amy Lowichik, M.D., Ph.D., professor pathology (pediatrics).

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