ADPKD primarily associates with the PKD1 gene [MIM#601313, chromosome 16p13.3] and/or the PKD2 gene [MIM#173910, chromosome 4q21] mutations,2,3 ascribing 85% and 15% of ADPKD patients, respectively.4 Stronger association with a severe clinical presentation and poorer prognosis have been reported for mutations in PKD1 than those in the PKD2 gene, suggestive of the critical role of genes in outcome prediction of patients with ADPKD.4 This evidence concerns the gene PKD2 and autosomal dominant polycystic kidney disease.