| Abstract: | The fasting serum concentrations of primary bile acids were determined in 30 patients with cystic fibrosis, aged 1 to 27 years, and correlated to liver disease. Cholic (fs-C) and chenodeoxycholic (fS-CDC) acids were determined by radioimmunoassays. Two patients had biopsy-proven liver cirrhosis, 13 had portal fibrosis. 8 had minor different pathological changes, and 7 had normal liver morphology. Standard liver function tests were of no help in evaluating liver disease in these patients. Eight patients had increased fS-C and 15 had increased fS-CDC, not correlated to liver morphology. Serum bile acid determination seems to be of no value in evaluating the extent of liver disease in cystic fibrosis. The more frequent and more marked increase of fS-CDC than of fS-C suggests that there is another hepatic clearance of bile acids in CF and/or that intestinal factors have a greater influence on the serum bile acid concentrations in this disease. |
