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Updated: Aug 17 2019

Maturity Onset Diabetes of the Young (MODY)

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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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