In fact, our patient with an LP variant in PKD1, KS, and a compound heterozygous GS-associated variant showed a severe phenotype of ADPKD, characterized by the development of early CRF (at 27 years old) and rapid evolution to ESRD (at 40 years old) (Table 1), associated with an intracranial aneurysm (Figure 1C). This evidence concerns the gene PKD1 and autosomal dominant polycystic kidney disease.