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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
 

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