glycodeoxycholic-acid and Cystic-Fibrosis

Topics: Bile Acids and Salts; Biomarkers; Cystic Fibrosis; Digestive System Diseases; Glycodeoxycholic Acid; Humans; Liver; Liver Diseases

Topics: Bile Acids and Salts; Biomarkers; Cystic Fibrosis; Digestive System Diseases; Glycodeoxycholic Acid; Humans; Liver; Liver Diseases

Cystic fibrosis (CF) and CF-related liver disease can lead to disturbances in bile acid metabolism.. This study determined serum bile acid concentrations in CF to define their usefulness in liver disease assessment.. Primary, secondary and conjugated bile acid levels were measured in three CF groups (25 patients each) exhibiting: liver cirrhosis, other liver disease, no liver disease, and in 25 healthy subjects (HS).. Bile acid levels were higher in CF patients than in HS, except for glycodeoxycholic acid (GDCA). However, bile acid concentrations did not differ between patients with cirrhosis and other liver involvement. GDCA and deoxycholic acid (DCA) differentiated CF patients with non-cirrhotic liver disease from those without liver disease (GDCA-AUC: 0.924, 95%CI 0.822-1.000, p<0.001; DCA-AUC: 0.867, 95%CI: 0.731-1.000, p<0.001). Principal component analysis revealed that in CF liver disease was related to GDCA, GGTP activity, severe genotype and pancreatic insufficiency.. A CF-specific bile acid profile was defined and shown to relate to liver disease. GDCA differentiates patients with non-cirrhotic liver involvement from those with no detectable liver disease. Hence, GDCA is a candidate for validation as a biomarker of non-cirrhotic progression of liver disease in CF.

Topics: Adolescent; Adult; Bile Acids and Salts; Biomarkers; Case-Control Studies; Cystic Fibrosis; Female; Glycodeoxycholic Acid; Humans; Liver Cirrhosis; Liver Diseases; Male; Young Adult

Measurements of serum bile acids (glycine conjugates of cholic, chenodeoxycholic, deoxycholic, and lithocholic acids) by radioimmunoassay in a variety of pediatric hepatobiliary disorders showed elevations in neonatal hepatitis syndromes, cholestasis, and hepatitis of extrahepatic or intrahepatic origin. Measurements of individual serum bile acids failed to differentiate between the various neonatal hepatitis syndromes. In one patient with cholestasis, the increased levels of bile acids observed returned to normal following therapy with cholestyramine and phenobarbital. In chronic active hepatitis the serum bile acid values correlated well with the bilirubin and SGOT in response to therapy with corticosteroids. These data confirm suggestions that serum cholylglycine and chenodeoxycholylglycine levels are a sensitive indicator of disturbed hepatic function and can be used in monitoring the course, activity, and therapeutic response in various hepatitis syndromes. In Reye's syndrome and protracted diarrhea of infancy, elevations in serum bile acids were detected without associated hyperbilirubinemia and provided additional evidence of disturbed hepatic function.

Topics: Adolescent; Bile Acids and Salts; Child; Child, Preschool; Cholestasis; Cystic Fibrosis; Diarrhea, Infantile; Glycochenodeoxycholic Acid; Glycocholic Acid; Glycodeoxycholic Acid; Hepatitis; Humans; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Diseases; Liver Diseases; Reye Syndrome