Observations on the pathophysiology of bartter's syndrome in childhood

A 20-month old boy with Bartter's syndrome was studied. Before treatment with indomethacin, he showed an abnormal response of blood pressure to Saralasin or angiotensin II infusion, elevated activity of the renin-angiotensin-aldosterone system, increased excretion of urinary kallikrein, and low distal fractional reabsorption of chloride. However, on receiving indomethacin, the boy's abnormalities returned to normal except for low distal fractional reabsorption of chloride on indomethacin. These findings suggest that a defect in chloride reabsorption in the ascending limb of Henle's loop may playa significant role in the pathogenesis of Bartter's syndrome