disease has basis in dysfunction of UMOD autosomal dominant medullary cystic kidney disease with or without hyperuricemia autosomal dominant medullary cystic kidney disease with hyperuricemia ADMCKD2 ADTKD-UMOD An inherited disorder that causes a gradual loss of kidney function that eventually leads to the need for kidney transplantation or dialysis between the ages of 30 and 70, which is caused by a mutation in the UMOD gene, which leads to the build-up of the altered uromodulin protein in the tubules of the kidney, leading to slow loss of kidney function. Patients with ADTKD-UMOD have high blood levels of uric acid before kidney failure develops, and some affected individuals may develop gout. GARD:0010679 ICD10:Q61.5 MONDO:0019511 Orphanet:88950 UMLS:CN206322 UMOD-related ADTKD UMOD-related autosomal dominant tubulointerstitial kidney disease autosomal dominant medullary cystic kidney disease type 2