Serum bile acids as markers of juvenile liver disease in alpha 1-antitrypsin deficiency.

Serum bile acids were studied in 34 patients, aged 7/12 to 20 years, with alpha 1-antitrypsin deficiency. Of these patients, 27 were of Pi-phenotype Z and 7 of SZ. Liver biopsy according to Menghini was performed in 19 patients. Fasting serum levels of cholic and chenodeoxycholic acids, determined by radioimmunoassay, were compared to conventional liver function tests and liver morphology. All patients with morphological liver cirrhosis had increased fasting levels of serum bile acids. The other patients, including those with severe fibrosis, had normal values. Although standard liver function tests were more pathological in the cirrhotic patients than in the others, serum fasting bile acids seemed to be the most distinct markers of severe liver disease.