Endogenous ursodeoxycholic acid and cholic acid in liver disease due to cystic fibrosis

Autor: Peter Lewindon, Anita C. Hoskins, Tamara N. Pereira, Ross W. Shepherd, Kenneth D.R. Setchell, J. L. Smith, Grant A. Ramm, Nancy C. O'Connell
Rok vydání: 2004
Předmět:
Zdroj: Hepatology (Baltimore, Md.). 39(6)
ISSN: 0270-9139
Popis: Focal biliary cirrhosis causes significant morbidity and mortality in cystic fibrosis (CF). Although the mechanisms of pathogenesis remain unclear, bile acids have been proposed as potential mediators of liver injury. This study examined bile acid composition in CF and assessed altered bile acid profiles to determine if they are associated with incidence and progression of liver injury in CF-associated liver disease (CFLD). Bile acid composition was determined by gas-liquid chromatography/mass spectrometry in bile, urine, and serum samples from 30 children with CFLD, 15 children with CF but without liver disease (CFnoLD), and 43 controls. Liver biopsies from 29 CFLD subjects were assessed histologically by grading for fibrosis stage, inflammation, and disruption of the limiting plate. A significantly greater proportion of endogenous biliary ursodeoxycholic acid (UDCA) was demonstrated in CFnoLD subjects vs. both CFLD subjects and controls (2.4- and 2.2-fold, respectively; ANOVA, P =.04), and a 3-4 fold elevation in endogenous serum UDCA concentration was observed in both CFLD subjects and CFnoLD subjects vs. controls (ANOVA, P
Databáze: OpenAIRE