The most common VUS overall were not KSD-linked, including polycystic kidney disease (PKD1, N = 21), familial Mediterranean fever (MEFV, N = 17), Fraser syndrome (FRAS1, N = 11), familial hyperaldosteronism type IV (CACNA1H), and Senior-Loken syndrome 4 (NPHP4, N = 10). The gene discussed is PKD1; the disease is nephronophthisis 4.