Liver involvement in cystic fibrosis. A report of 9 cases

9 out of 204 unselected cystic fibrosis (CF) patients seen at the Department of Pediatrics, University of Berne, Switzerland, over the last 20 years had clinically overt liver disease. In 7 patients liver cirrhosis was demonstrated (3.4%). Focal biliary cirrhosis was the pertinent finding in 3 cases, whereas another 3 showed unspecific nodular cirrhosis. One infant presented with prolonged obstructive jaundice due to partial extrahepatic atresia and delayed passing of meconium. Two other patients had steatosis. A review of the literature dealing with clinical and laboratory findings in CF patients with liver disease is given.