UMOD and autosomal dominant medullary cystic kidney disease with or without hyperuricemia: It is genetically heterogenous with pathogenic variants in MUC1,3REN,4HNF1B5 and uromodulin (UMOD)6 identified, with UMOD comprising the highest proportion of ADTKD cases of up to 50%, with a disease prevalence of 9 per million.7 ADTKD-UMOD is estimated to account for 2% of patients with kidney failure,8 with susceptibility UMOD variants also conferring around 20% increased risk for CKD and 15% for hypertension.9 Classically, it is characterised by early onset gout, hyperuricaemia, the absence of haematuria or proteinuria and kidney failure usually occurring between 30 and 60 years.