In humans, there are a variety of different manifestations of CF hepatobiliary disease; most commonly CFTR dysfunction associates with focal biliary cirrhosis (Blanc & Di Sant'Agnese, 1956; Colombo, 2007; Colombo et al., 2002; Leung & Narkewicz, 2017; Oppenheimer & Esterly, 1975a, 1975b; Vawter & Shwachman, 1979). This evidence concerns the gene CFTR and cystic fibrosis.