ABSTRACT

Although pulmonary disease remains the major cause of morbidity and mortality in cystic brosis (CF), cystic brosis-associated liver disease (CFALD) is becoming an increasingly important clinical issue. In some cases, it may even be a dominant manifestation. Liver disease is the most important non-pulmonary cause of death in CF patients, accounting for an estimated 2.5% of overall mortality.1 CFALD also represents a more severe phenotype in CF and may be an independent risk factor for mortality.2