Ultrasound Assessment of the Liver in Patients with Cystic Fibrosis

Abstract

Cystic fibrosis (CF) is a genetic multi-system disorder in which there is an underlying defect in the transport of ions across cell membranes. A variety of mutations in the CF transmembrane regulator (CFTR) gene result in a failure of chloride channel activity in epithelial tissues.^[1,2] Clinical effects are manifest in lung, pancreas, liver, gastrointestinal tract and the male reproductive system. In the past, the pulmonary manifestations have predominated in terms of morbidity and mortality. However, the treatment of progressive lung disease in CF has improved greatly in recent years, with a resultant increase in patient life expectancy. This has lead to an increased focus on evaluating and delaying disease-related damage to other organs^[3].