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