Urinary excretion of endothelin-1 (ET-1) and plasma ET-1 were measured in 21 children with absorptive idiopathic hypercalciuria (AIH) and 22 controls. The absorptive type of idiopathic hypercalciuria was determined by a calcium loading test. Daily urinary excretion of ET-1 and urinary ET-1/creatinine ratio were significantly increased ( P=0.005 and P=0.007, respectively) in patients with AIH (9274+/-6444 pg/24 h and 14.04+/-9.52 pg/mg, respectively) compared with controls (4699+/-2120 pg/24 h and 7.36+/-4.71 pg/mg, respectively). Plasma ET-1 levels were significantly lower in patients with AIH (0.84+/-0.64 pg/ml) than in controls (1.54+/-0.54 pg/ml, P=0.0001). In conclusion, patients with AIH had increased urinary ET-1 excretion and decreased plasma ET-1 levels. This is most likely due to the decreased reabsorption of ET-1 in the renal tubule and increased renal production.