The survival of 85 anti-mitochondrial antibody (AMA)-positive (mean Mayo risk score, 5.11) and 19 AMA-negative (mean Mayo risk score, 4.77) primary biliary cirrhosis patients, under ursodeoxycholic acid not subjected to liver transplantation, was compared with the estimated survival of a simulated control group of untreated patients created with the updated Mayo model and a control group from the general population. In the first 7 years 3 AMA-negative patients died, versus 12 under the Mayo model (P = 0.01), and 10 AMA-positive patients, versus 26 under the Mayo model (P < 0.005), with 7 expected deaths from the general population (P < 0.0001). At 10 years the cumulative survival differed in the treated patients overall (P < 0.0001) but not in the early primary biliary cirrhosis (stages I-II) patients compared to the general population. Therefore the survival of our patients treated with ursodeoxycholic acid is higher than that predicted from the Mayo model. Early treatment may prolong survival.

SUMMARYBackground: Endothelins and nitric oxide regulate sinusoidal blood flow and the perfusion of the peribiliary vascular plexus. Aims: To study the serum and hepatic vein concentration of ET-1, ET-2, ET-3 and nitric oxide in patients with primary biliary cirrhosis and the effect of ursodeoxycholic acid treatment. Methods: Endothelins and nitrites/nitrates were measured in serum and hepatic vein blood in primary biliary cirrhosis and viral cirrhotic patients prior and after ursodeoxycholic acid therapy and in serum in controls. Endothelins were measured with commercial enzymelinked immunosorbent assays and nitrites/nitrates with a modification of Griess reaction. Results: The ET-1 and ET-3 levels were similar in patients and controls. Primary biliary cirrhosis patients

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