Although bile duct paucity has been identified in almost all adult patients with ALGS and hepatic involvement reported in the literature [8–10, 54, 104, 105], it can also be found in patients with primary sclerosing cholangitis, primary biliary cholangitis, cystic fibrosis liver disease, alpha-1 antitrypsin deficiency, and drug induced liver injury [106, 107]. Here, SERPINA1 is linked to primary biliary cholangitis.