The 2015 Kidney Disease: Improving Global Outcomes (KDIGO) guideline systematically addresses autosomal dominant tubulointerstitial nephropathy (2); five ADTKD-causing genes (MUC1, UMOD, REN, HNF1B, and SEC61A1) and their corresponding genotypes have been identified (3). This evidence concerns the gene SEC61A1 and autosomal dominant medullary cystic kidney disease with or without hyperuricemia.