A major multi-center investigation of children with cystic fibrosis has identified a test that allows earlier identification of those at risk for cystic fibrosis liver disease.
The study, which includes 11 clinical sites in North America, was led by Michael Narkewicz, MD, professor of pediatrics from the University of Colorado School of Medicine and Children's Hospital Colorado. The findings of the study are published today ahead of print in The Journal of Pediatrics .
Cystic fibrosis is a genetic disorder that primarily affects the lungs and the pancreas, but can also create problems in the liver and other organs. Advanced cystic fibrosis liver disease refers to advanced scarring of the liver, which occurs in about 7 percent to 10 percent of patients with cystic fibrosis. The exact cause of advanced cystic fibrosis liver disease is not known, but only individuals with cystic fibrosis develop it.
As we develop new therapies for cystic fibrosis and for other liver diseases, it is critical that we better understand which patients with cystic fibrosis are at higher risk for cystic fibrosis liver disease. Our study is a vital first step in identifying those patients and should help in the creation of targets for therapies that could prevent cystic fibrosis liver disease." Michael Narkewicz, MD, professor of pediatrics from the University of Colorado School of Medicine and Children's Hospital Colorado
The results reported in the article show that researchers could identify children at higher risk for advanced liver disease by using research-based ultrasound screening with consensus grading by four radiologists. The report is a four-year interim analysis conducted as part of a projected nine-year study. Related Stories
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