We measured plasma concentration of endothelin-1 in three children wit
h Byler's disease, five with biliary atresia after portoenterostomy, a
nd nine controls. No patients had ascites or hepatorenal syndrome. Pla
sma endothelin-1 levels were significantly higher in patients with Byl
er's disease than in the controls (5.19 +/- 0.90 versus 1.81 +/- 0.19
pg/ml, respectively; p < 0.01), but were normal in operated biliary at
resia. Urinary concentrations of N-acetyl-beta-D-glucosaminidase (NAG)
were significantly higher in the patients with Byler's disease than i
n controls. Plasma endothelin-1 level correlated significantly with se
rum concentration of bile acid (r = 0.91; p < 0.01) and urinary concen
tration of NAG (r = 0.92; p < 0.01). We conclude that plasma endotheli
n-1 levels are high in patients with severe biliary cirrhosis and that
endothelin-1 may partially contribute to development of renal injury
in cirrhosis.