In 2015, the KDIGO guidelines standardized the terminology as “ADTKD” (Eckardt et al., 2015) and established a molecular subtyping system based on causative genes (ADTKD-UMOD, -REN, -HNF1B, -MUC1, and -SEC61A1). The gene discussed is HNF1B; the disease is autosomal dominant medullary cystic kidney disease with or without hyperuricemia.