REN and autosomal recessive disease: BS is an autosomal recessive disease, with an incidence of 1.2 per million.[1] Classic BS described by Bartter in 1962 is the type III disease associated with defect in ion transport in the thick segment of the ascending loop of Henle and distal convoluted tubule.[2, 3] This results in increased urinary loss of potassium and hypokalemic metabolic alkalosis with hypercalciuria.[4] Loss of sodium and chloride stimulates the renin-angiotensin-aldosterone axis.