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Insulin autoimmune syndrome

Disease definition

A rare endocrine disease characterized by hyperinsulinemic hypoglycemia associated with the presence of autoantibodies to endogenous insulin without previous exposure to exogenous insulin. Patients usually present in adulthood with postprandial, fasting-, or exercise-induced hypoglycemia, often with pronounced neuroglycopenic symptoms. Laboratory investigations reveal markedly elevated serum insulin, as well as increased C-peptide and proinsulin. The condition may be associated with other autoimmune diseases, monoclonal gammopathy, and/or recent exposure to certain medications.

ORPHA:411593

Classification level: Disorder
  • Synonym(s):
    • Hirata disease
  • Prevalence: Unknown
  • Inheritance: Not applicable 
  • Age of onset: Adult, Elderly
  • ICD-10: E16.1
  • OMIM: -
  • UMLS: C0854359
  • MeSH: -
  • GARD: 10808
  • MedDRA: -
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