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Addison Disease

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Topic updated on 11/17/16 12:20pm

Introduction
  • A disorder caused by the destruction of the adrenal cortices
    • involves all three layers of the cortex
    • results in ↓ production of glucocorticoid (cortisol), mineralcorticoids (aldosterone), and steroids
  • Autoimmune destruction accounts for 80% of the spontaneous cases
    • other causes include:
      • congenital enzyme deficiencies
      • TB
      • AIDS
      • metastasis
  • Associated with other autoimmune conditions
Presentation
  • Symptoms  
    • fatigue worsened by stress
    • weakness
    • weight loss
    • nausea and vomitting
  • Physical exam
    • increased skin pigmentation
      • due to melanocyte stimulating hormone, a by-product of ↑ ACTH
        • ACTH/MSH both arise from pro-opiomelanocortin (POMC)
      • most commonly seen in mouth
    • hypotension
      • due to ↓ production of mineralcorticoids (aldosterone)
        • results in hyponatremic volume contraction
Evaluation
  • Labs
    • must determine if ↓ production of cortisol is primary (of adrenal orgin) or secondary (of pituitary origin)
      • ↑ plasma ACTH (primary)
      • plasma ACTH (secondary)
    • ACTH stimulation test
      • low cortisol and 17-OH levels in response
    • metyrapone test
      • ↑ ACTH without ↑ in 11-deoxycortisol
    • hyponatremia and hyperkalemia
      • due to ↓ aldosterone that results in decreased sodium reapsorption and decreased potassium secretion
    • hypoglycemia
      • due to ↓ cortisol-stimulated gluconeogenesis
    • metabolic acidosis
      • due to bicarbonate
    • eosinophilia, lymphocytosis, and neutropenia
      • due to ↓ cortisol
        • normally suppresses levels of eosinophils and lymphocytes
        • normally de-marginates a portion of the neutrophil population which results in ↑ measurable neutrophil count
Treatment
  • Replace
    • glucocorticoids
    • mineralocorticoids
  • Administer stress dose steroids at time of stress (surgery or significant illness)


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Qbank (1 Questions)

TAG
(M1.EC.32) A 27-year-old Caucasian female presents complaining of recent weight loss and weakness. She reports that she feels dizzy and lightheaded every morning when she gets out of bed, and often at work whenever she must rise from her desk. Physical exam reveals several areas of her skin including her elbows and knees are more pigmented than other areas. Which of the following would be consistent with the patient's disease? Topic Review Topic

1. Hyperglycemia
2. Hyperkalemia
3. Hypernatremia
4. Central obesity
5. Pretibial myxedema

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