Abstract
The childhood forms of polycystic kidney disease are autosomal recessive polycystic kidney disease and nephronophthisis. In animal models of these diseases, characterised by a defect in urine concentration progressing to renal failure, the selective vasopressin receptor type 2 antagonist OPC-31260 halted the progression or even caused regression of the established disease. Vasopressin receptor type 2 antagonists should proceed to clinical trial for these polycystic kidney diseases.