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We measured plasma concentration of endothelin-1 in three children with Byler's disease, five with biliary atresia after portoenterostomy, and nine controls. No patients had ascites or hepatorenal syndrome. Plasma endothelin-1 levels were significantly higher in patients with Byler'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 atresia. Urinary concentrations of N-acetyl-β-L-glucosaminidase (NAG) were significantly higher in the patients with Byler's disease than in controls. Plasma endothelin-1 level correlated significantly with serum concentration of bile acid (r= 0.91; p< 0.01) and urinary concentration of NAG (r= 0.92; p< 0.01). We conclude that plasma endothelin-1 levels are high in patients with severe biliary cirrhosis and that endothelin-1 may partially contribute to development of renal injury in cirrhosis.

To determine the relationship between low magnesium status and lipids, we divided 27 patients with microscopic hematuria and normal renal function into two groups according to magnesium retention, as measured by a magnesium-loading test, and compared their serum lipid and apolipoproteins. Patients with low magnesium status (n = 7) had significantly lower levels of cholesterol, HDL cholesterol, and apolipoprotein A-1 than those with normal magnesium status (n = 20); however, there were no significant differences between the groups in serum concentrations of magnesium and apolipoprotein B. These data suggest that magnesium deficiencies are associated with low serum concentration of HDL cholesterol and apolipoprotein A-1.