Related Experiment Videos
Abnormal biliary lipid composition in cystic fibrosis. Effect of pancreatic enzymes
Insights
Bile in cystic fibrosis patients is more likely to form gallstones when pancreatic enzymes are not taken. Pancreatic enzyme therapy normalizes bile composition, reducing gallstone risk.
Area of Science:
- Gastroenterology
- Hepatology
- Pediatric Medicine
Background:
- Cystic fibrosis (CF) is associated with a higher incidence of gallstones.
- Biliary lipid composition plays a crucial role in gallstone formation.
Purpose of the Study:
- To compare biliary lipid composition in patients with cystic fibrosis (CF), children with cholelithiasis, and healthy controls.
- To investigate the effect of pancreatic enzyme therapy on bile composition in CF patients.
Main Methods:
- Biliary lipid composition was analyzed in 26 CF patients, 7 children with cholelithiasis, and 13 controls.
- CF patients were studied both with and without pancreatic enzyme therapy.
- Cholesterol saturation index and bile acid profiles were assessed.
Main Results:
- Bile from CF patients not taking pancreatic enzymes showed increased cholesterol and a higher saturation index, comparable to patients with gallstones.
- Pancreatic enzyme therapy in CF patients normalized cholesterol levels and saturation index, similar to controls.
- CF patients exhibited a different bile acid profile (higher cholic/chenodeoxycholic acid ratio) compared to controls and cholelithiasis groups, irrespective of enzyme therapy.
Conclusions:
- Bile in untreated cystic fibrosis is lithogenic, indicating a predisposition to gallstone formation.
- Pancreatic enzyme therapy effectively modifies bile composition in CF patients, potentially reducing gallstone risk.
Abstract:
Because of the increased incidence of gallstones in cystic fibrosis we compared biliary lipid composition in 26 patients with cystic fibrosis, seven children with cholelithiasis but no cystic-fibrosis and 13 controls. Eighteen of the cystic fibrosis group had cholecystograms, and only one had gallstones. In 14 patients with cystic fibrosis who had stopped taking pancreatic enzymes for one week molar percentage of lipid composition accounted for by cholesterol (mean +/- S.E., 16.3 +/- 2.9) and saturation index (2.0 +/- 0.3) were comparable to values of the cholelithiasis group and higher (P less than 0.01) than those of controls. In 12 patients with cystic fibrosis taking pancreatic enzymes, molar percentage of cholesterol (8.6 +/- 1.7) and saturation index (1.0 +/- 0.1) did not differ from those of controls; in cystic fibrosis there was a preponderance of cholic over chenodeoxycholic acid both off (1.7 +/- 0.2) and on (1.9 +/- 0.3) therapy as compared to the cholelithiasis (0.7 +/- 0.1) and control (0.8 +/- 0.0) groups. The glycine/taurine ratio of conjugated bile acids were lower in enzyme-treated patients with cystic fibrosis (3.7 +/- 0.6) than in patients off treatment (6.4 +/- 1.0), but was higher (P less than 0.01) than in controls (1.8 +/- 0.2). Bile is lithogenic in untreated cystic fibrosis and responds to pancreatic enzymes.