PKD2 and autosomal dominant polycystic kidney disease: In an analysis of 42 VEO-ADPKD cases targeting PKD1, PKD2, HNF1B and PKHD1, 37.2% of patients harbored additional PKD-related variants inherited from an asymptomatic parent in addition to the primary pathogenic variant—a significantly higher frequency than the 14.4% observed in adult-onset cases9.