Gastrointestinal Manifestations in Cystic Fibrosis

Abstract Purpose of Review Cystic fibrosis (CF) has a significant impact on the luminal gastrointestinal (GI) tract, pancreas, and hepatobiliary system. The aim of this review is to highlight the broad spectrum of gastrointestinal manifestations of CF, and to provide key information regarding screening, diagnosis, and management, with particular emphasis on existing clinical care guidelines. Recent Findings The review highlights the impact of highly effective cystic fibrosis transmembrane conductance regulator (CFTR) modulators on GI manifestations of CF as well as the recent change in nomenclature and clinical care guidelines for CF hepatobiliary disease. Summary A vast array of luminal GI, pancreatic and hepatobiliary pathophysiologic conditions may occur in people with CF which may inform screening and diagnostic evaluation. Additionally, nonspecific GI symptoms are highly prevalent and are largely unchanged in the highly effective modulator era. An understanding of CF pathophysiology, current therapies and key clinical guidelines are essential in the care of people with CF.