Endocrinology Book

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Addison's DiseaseAka: Addisonian Crisis, Adrenal Cortical Insufficiency, Adrenal Insufficiency, Adrenal Hypofunction, Illness Induced Adrenal Insufficiency, Stress Dose Steroid

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  1. See Also
    1. Autoimmune Polyglandular Syndrome
  2. Pathophysiology
    1. Hypothalamic-Pituitary-Adrenal Axis (HPA) Dysfunction
      1. Lab features: Cortisol low, ACTH Low
      2. Causes
        1. Severe illness
        2. Exogenous Corticosteroid (suppression lasts months)
          1. Prednisone >7.5 mg/day for >3 weeks
          2. Dexamethasone >0.75 mg/day for >3 weeks
        3. Pituitary Infarction
        4. CNS Infection or tumor invasion
        5. Head Injury
        6. HIV Infection
    2. Adrenal Insufficiency
      1. Lab features: Cortisol low, ACTH high
      2. Causes
        1. Addison's Disease
          1. Primary adRenal Failure of adrenal glands
        2. Idiopathic adrenal hypoplasia or destruction
          1. Granulomatous disease (e.g. Tuberculosis, Fungus)
          2. Amyloidosis
          3. Hemochromatosis
          4. Tumor
          5. Autoimmune process
        3. Medication medicated cortisol inhibition
          1. Etomidate
          2. Ketoconazole
  3. Symptoms
    1. Weakness
    2. Fatigue or Lethargy
    3. Nausea or Vomiting
    4. Diarrhea
    5. Abdominal Pain
    6. Weight loss
    7. Salt craving
    8. Altered Level of Consciousness (e.g. Delirium)
  4. Signs
    1. Fever
    2. Hyperpigmentation
      1. Skin with mottled areas of increased pigmentation
      2. Pigment at Buccal mucosa (Caucasian)
      3. Increased pigment in lips, vagina, rectum
    3. Reduced Hair Growth
    4. Postural Hypotension
    5. Signs of dehydration
  5. Labs
    1. Complete Blood Count
      1. Normochromic Normocytic Anemia
      2. Eosinophilia (helps differentiate from other causes)
    2. Chemistry Panel
      1. Hyponatremia
      2. Hypochloremia
      3. Hyperkalemia
      4. Hypoglycemia (helps differentiate from other causes)
    3. Urine 17-ketosteroids low
    4. Urine 17-Hydroxysteroids low
  6. Diagnosis
    1. Corticotropin Stimulation Test
      1. Failed adrenal response
  7. Management
    1. Acute
      1. IV Cortisol
    2. Chronic Replacement
      1. Hydrocortisone (glucocorticoid) AND
      2. Mineralocorticoid (9-alpha-fluorocortisol)
    3. Stress Dose Steroid
      1. Indication: Acute severe illness
        1. Serum Cortisol <15 to 34 mcg/dl
        2. Cortisol <9 after Corticotropin Stimulation Test
      2. Management
        1. Hydrocortisone 50 mg IV or IM q6 hours
        2. Consider adding fludriCortisone 50 mcg qd
  8. Resources
    1. Addison and Cushing International Federation (ACIF)
      1. http://www.nvacp.nl/page.php?main=5
  9. References
    1. Cooper (2003) N Engl J Med 348:727

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