ABSTRACT
I. Introduction Cystic fibrosis-associated liver disease (CFALD) is a serious complication with significant morbidity and mortality for patients with cystic fibrosis (CF), as well as a vexing entity that continues to challenge the best of scientists and clinicians. Although involvement of the liver and biliary tract has been recognized as a complication of CF since Andersen’s initial description in 1938 (1), the clinical significance of hepatobiliary disease in CF is not well characterized. This is due to the fact that liver involvement in CF is often asymptomatic, though silent in nature, surfacing only once end-stage complications of liver disease. Historically, the pulmonary complications of CF were associated with early mortality and therefore often eclipsed the liver involvement. Although pulmonary disease still accounts for the greatest mortality from CF, CFALD is now considered the third leading cause of death from CF (after pulmonary and transplant complications) (2). Fundamental questions remain however regarding the pathogenesis, detection, natural history, and treatment of CFALD as highlighted in this chapter.
