Hepatic failure, cardiac decompensation, hypercholesterolaemia with early development of atherosclerosis are risks which endanger children with Alagille’s syndrome. High total and LDL cholesterol concentrations, lower HDL concentration, changes of apolipoproteins are found in serum of these children already during their first year of life. After short-term pravastatin administration (HMG-CoA reductase inhibitor) a marked reduction of the LDL cholesterol concentration occurred as well as of total cholesterol, triacyglycerols, apolipoprotein B and lipoprotein (a). During this period the HDL cholesterol concentration increased as well as apolipoprotein A-I. 5 mg pravastatin/day proved effective and safe. Lower doses - 2.5 mg/day had not therapeutic effect. During the subsequent 6 months when the child was only on a diet and took Ursosan (ursodeoxycholic acid) practically all investigated parameters of the lipid metabolism returned to baseline values recorded at the onset of pravastatin therapy. The HDL concentration remained however permanently above 1 mmol/l, the apolipoprotein B value permanently lower. The lipoprotein(a) concentration increased again. Toxic damage of already damaged livers was not observed. The short-term ratio of both transaminases (AST >> ALT) returned to original levels, their total concentrations being lower ALT = 1.49 mkat/l, AST = 1.30 mkat/l.

        Key words: statins, hypercholesterolaemia, Alagille’s syndrome, test