Four patients with cystic fibrosis developed prolonged obstructive jaundice starting in the newborn period. Obstructive biliary cirrhosis was shown post mortem in one of them who died at 5 months from pneumonia, while another dying at 8 years had an histologically normal liver at necropsy. The two survivors were jaundiced for 6 months and 5 weeks respectively, before making a clinical recovery, and in both liver biopsy at the height of the jaundice showed bile stasis.
Meconium ileus was present in half of all recorded cases of cystic fibrosis with prolonged neonatal jaundice. Jaundice is probably due to extrahepatic biliary obstruction from bile of increased density, with secondary intrahepatic bile stasis.