Additionally, referrals for assessment and evaluation of extrarenal features may allow for proactive management.10, 11, 12, 13, 14 In recognition of the potential utility gained with identifying molecular etiology, the recent Kidney Disease: Improving Global Outcomes (KDIGO) guideline suggests incorporating the causal gene into the disease nomenclature for individuals diagnosed with ADPKD and have a pathogenic variant in PKD1, PKD2, or a gene for which pathogenicity is well supported (eg, ADPKD-PKD1).15 The gene discussed is PKD2; the disease is autosomal dominant polycystic kidney disease.