Cystic fibrosis (CF) results from autosomal recessive mutations in the CFTR gene and is characterized by abnormal chloride and sodium transport with consequent thick, viscous secretions. In the liver, bile ducts may be obstructed by abnormal mucoid secretions or inspissated bile. Progressive liver injury results in cirrhosis, as seen here, with macronodules (>3 mm). Liver disease is the third most common cause of death in CF and transplantation is the only effective treatment. Image Copyright: pathorama.ch.