PKD1 and autosomal dominant polycystic kidney disease: ADPKD itself is a risk factor for IA formation in the general population9,10, and mutation in PKD1 and PKD2 and its genotypes affect total kidney volume (TKV)16, kidney disease severity17–21, and IA22,23, which suggests that factors associated with ADPKD, including mutations in PKD1 and PKD2, TKV, and kidney function are also probable risk factors for IA in patients with ADPKD.