HNF1B and renal cysts and diabetes syndrome: Monoallelic loss-of-function mutations in HNF1β have also been associated with several abnormalities of variable penetrance such as CAKUT, renal cysts, diabetes [mature-onset diabetes of the young type 5 (MODY-5)], elevated liver enzymes, neurological/cognitive/psychiatric disorders, delayed psychomotor development, and miscellaneous syndromic features (549).