With 79% PKD1 carriers (including 9% PKD1-VUS carriers) and 11% PKD2 carriers, the results of our investigation reflect the genetic distribution found in larger ADPKD cohort studies.3,23 Importantly, genotypic differences in renal survival confirmed previous reports3,4,24 although PKD2 patients appeared more severely affected, underlined by the high proportion of polycystic liver disease among PKD2 carriers in our cohort (92%). Here, PKD2 is linked to Isolated polycystic liver disease.