Although inheritance was more likely to be AD in adults, the majority (16/21, 75%) still had extrarenal associations, including the most common genetic causes of end-stage kidney failure (XL Alport syndrome, AD polycystic kidney disease and AD tubulointerstitial kidney disease -UMOD or -HNF1B).14, 15, 16,18, 19, 20. Here, HNF1B is linked to familial juvenile hyperuricemic nephropathy.