Introduction Diabetic symptoms as result of genetic mutations in metabolic transcription factor genes many different loci have been identified often involve glucose-sensing mechanisms in the pancreas AD inheritance Patients typically < 25 years No association with obesity No defect in insulin sensitivity - only insulin release Evaluation Serology mild to severe hyperglycemia do not see ketosis Treatment Medical sulfonylureas insulin adjunctive diabetic agents Prognosis, Prevention, and Complications May progress to type II DM