HNF1B and cystic kidney disease: Beyond its critical function in kidney development, HNF-1β also serves as a transcription factor with broad implications, regulating gene expression in the epithelial cells of several other vital organs, including the pancreas, the paramesonephric duct and liver.[17,18] This condition exhibits a broad clinical spectrum of renal developmental anomalies, encompassing renal cysts, GCKD, and solitary kidneys.