AVPR2 and autosomal dominant polycystic kidney disease: While the administration of the selective vasopressin V2 receptor (V2R) antagonist, tolvaptan, is considered the gold standard treatment for ADPKD [40,41], clinical practice recommendations for the treatment of individuals with AS encourage the early use of angiotensin-converting enzyme (ACE) inhibitors that have proven efficient to reduce lifetime risk for ESRD [42,43].