Maturity Onset Diabetes of the Young (MODY)

Introduction

image 110
  • 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