Endocrinology Book

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Cushing's DiseaseAka: Cushing's Syndrome

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  1. Etiologies
    1. Iatrogenic
      1. Steroid therapy (most common cause)
    2. Central Cause
      1. Pituitary adenoma
    3. Adrenal Cause
      1. Adrenal Adenoma
      2. Adrenal Hyperplasia
      3. Adrenal Malignancy (15%)
    4. Ectopic Source
      1. Malignancy (Small Cell Carcinoma of the lung: 15%)
  2. Symptoms
    1. Mood changes (depression and euphoria)
    2. Easy bruising
    3. Weakness
    4. Weight gain
    5. Amenorrhea
    6. Back pain
  3. Signs
    1. Truncal Obesity (90%)
    2. Hypertension (85%)
    3. Glucose Intolerance (80%)
    4. Hirsutism (70%)
    5. Wide, purple abdominal and thigh striae (65%)
    6. Osteoporosis (55%)
    7. Moon facies
    8. Buffalo hump (Thoracic kyphosis)
    9. Myopathy
    10. Plethoric face
    11. Supraclavicular fat pad development
    12. Hypertrichosis
    13. Peripheral Edema
    14. Hypertension
  4. Labs
    1. Screening Test
      1. 24-hour Urinary free cortisol level (preferred)
        1. Urine 17-Ketosteroid excretion
        2. Urine 17-Hydroxysteroid excretion
      2. Serum Cortisol
      3. Low dose Dexamethasone Suppression Test
        1. Dexamethasone 1 mg at 11pm
        2. Plasma Cortisol in following 8 AM
      4. Night-time Salivary cortisol testing
    2. Distinguish between pituitary, adrenal or ectopic cause
      1. Plasma ACTH
      2. High dose Dexamethasone Suppression Test (8 mg)
  5. Radiology
    1. CT or MRI Cone down Sella Turcica
      1. Pituitary adenoma
    2. CT abdomen
      1. Adrenal tumor
  6. Management
    1. Exogenous Cushing's
      1. Stop steroids or decrease dose
      2. Change steroid dosing to qod with drug holiday
    2. Endogenous Cushing's
      1. Surgically excise adenoma (in pituitary or adrenal)
  7. Resources
    1. Addison and Cushing International Federation (ACIF)
      1. http://www.nvacp.nl/page.php?main=5
  8. References
    1. Orth (1995) N Engl J Med 332:791

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