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. 