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Updated: Aug 22 2017

Addison Disease (Adrenal Insufficiency)

Snapshot
  • A 15-year-old male presents to his pediatrician with complaints of fatigue, weight loss, and recurrent nausea and vomitting.  On physical exam he appears weak and has skin that appears abnormally tan.  A basic metabolic panel reveals hyponatremia and hyperkalemia.
Introduction
  • A disorder caused by the destruction of the adrenal cortices
  • Autoimmune destruction accounts for 80% of the spontaneous cases in the U.S. 
  • Other causes include
    • congenital enzyme deficiencies
    • hemorrhage (Waterhouse-Friderichsen)
    • TB  
    • other infections
  • Leads to loss of cortisol, mineralcorticoids (aldosterone), and catecholamines
  • May be isolated or be a component of a polyglandular autoimmune syndrome
Presentation
  • Symptoms
    • fatigue worsened by stress
    • weakness
    • weight loss
    • nausea and vomitting
  • Physical exam
    • increased skin pigmentation (due to elevated pro-opiomelanocortinin MSH and ACTH) 
    • hypotension
Evaluation
  • Labs
    • elevated plasma ACTH
    • low cortisol levels in response to ACTH stim test 
    • decreased aldosterone leads to
      • hyponatremia
      • hyperkalemia
      • hypoglycemia
      • increased BUN & Cr
      • metabolic acidosis
      • eosinophilia
Differential
  • Anorexia nervosa, malabsorption states, occult malignancy, hypoparathyroidism, thyrotoxicosis, panhypopituitaryism
Treatment
  • Replace
    • glucocorticoids (prednisone)
    • mineralocorticoids (fludrocortisone)
  • Administer stress dose steroids at time of stress(surgery)
Question
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