These diseases including ‘familial juvenile hyperuricemic nephropathy (FJHN)’, ‘medullary cystic kidney disease (MCKD) type 2’, ‘uromodulin-associated kidney disease (UAKD)’, UMOD-related diseases, and ‘MCKD type 1’, suggested the adoption of new terminology using a single name ‘autosomal dominant tubulointerstitial kidney disease’ (ADTKD) by KDIGO [46]. Here, UMOD is linked to autosomal dominant medullary cystic kidney disease with or without hyperuricemia.