/renal cysts and diabetes syndrome

renal cysts and diabetes syndrome

Rare Disease

MONDO:0007669

Also Known As

CAKUT with diabetesHNF1B-MODYHNF1B-related renal cysts and diabetes syndromeMODY5RCADRCAD syndromeatypical FJHNatypical familial juvenile hyperuricemic nephropathycongenital anomalies of the kidney and urinary tract with diabetesfamilial hypoplastic glomerulocystic kidneyhepatocyte nuclear Factor 1-beta-associated monogenic diabeteshypoplastic type glomerulocystic kidney diseasematurity onset diabetes of the Young, type 5renal cysts and diabetes syndromerenal cysts-maturity-onset diabetes of the young syndromerenal dysfunction-early-onset diabetes syndromeFJHN atypicalFJHN, atypicalMODY type 5glomerulocystic kidney disease, hypoplastic typeglomerulocystic kidney, familial hypoplastichyperuricemic nephropathy, familial juvenile, atypicalmaturity-onset diabetes of the Young, type 5maturity-onset diabetes of the young type 5

Definition

Renal cysts and diabetes syndrome (RCAD) is a rare form of maturity-onset diabetes of the young (MODY) characterized clinically by heterogeneous cystic renal disease and early-onset familial non-autoimmune diabetes. Pancreatic atrophy, liver dysfunction and genital tract anomalies are also features of the syndrome.