HNF1B and hyperinsulinemic hypoglycemia, familial, 4: In particular, patients with HNF1B deficiency typically presented renal hypoplasia and agenesia, familial juvenile hyperuricemic nephropathy, glomerulocystic kidney disease, and renal interstitial fibrosis, eventually leading to chronic kidney disease [23,31,33,34,35,36,37,38,39].