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Cushing syndrome due to macronodular adrenal hyperplasia

Disease definition

ACTH-independent macronodular adrenal hyperplasia (AIMAH) is a rare cause of Cushing syndrome (CS; see this term) characterized by nodular enlargement of both adrenal glands (multiple nodules above 1 cm in diameter) that produce excess cortisol and features of adrenocorticotropic hormone (ACTH) independent CS (see this term).

ORPHA:189427

  • Synonym(s):
    • Primary bilateral macronodular adrenal hyperplasia
  • Prevalence: <1 / 1 000 000
  • Inheritance: Autosomal dominant or Not applicable 
  • Age of onset: All ages
  • ICD-10: E24.8
  • OMIM: 219080  615954
  • UMLS: -
  • MeSH: -
  • GARD: 10824
  • MedDRA: -

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