Cholelithiasis as the first manifestation of cystic fibrosis of the pancreas (mucoviscidosis)
Goriup, U.; Rickham, P.P.; Deyhle, P.; Shmerling, D.H.
Helvetica Paediatrica Acta 35(2): 177-184
1980
ISSN/ISBN: 0018-022X PMID: 7451232 Document Number: 159292
A hitherto healthy 6-year-old girl developed cholestatic jaundice, due to multiple extra- and intrahepatic cholesterol bile calculi. Cystic fibrosis of the pancreas was established as the cause of the disease and of the biliary cirrhosis. Operative treatment and the ensuing substitution with pancreatic extracts have improved the cholestasis. Pulmonary involvement became subsequently evident and is now under treatment. The pathophysiology of bile acids in cystic fibrosis as relevant to the occurrence of gall stones in these patients is discussed.