Liver Disease in Children with Cystic Fibrosis

Cystic Fibrosis-related Liver Disease (CFLD) is highly prevalent in the first decade of life. Nearly 10% of children with Cystic Fibrosis will develop cirrhosis and 1-3% of those with cirrhosis will require liver transplant. The purpose of this review article is to examine the current diagnostic criteria and propose options for new diagnostic criteria based on recent studies. Current diagnostic criteria are based on data from 1999 and does not discuss recommendations for imaging studies for CFLD screening. Imaging can help provide early detection of CFLD when ordered promptly and not delayed by waiting for abnormal physical exam findings or extremely high liver function tests. This article will also discuss current management and complications of CFLD. This author suggests utilizing ultrasound and/or fibroscan as a screening tool along with physical exam and laboratory testing for CFLD for all Cystic Fibrosis patients ages 5-15 on an annual to biannual basis, dependent upon findings