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v   = Addison’s disease = Adrenocortical insufficiency (failure).

v   Uncommon

v   ↓ All cortical hormones & ­ ACTH.

 

Causes

 

1) Autoimmune adrenalitis  (90%):

Organ-specific auto-Abs + lymphocytes    à destruction of adrenal cortex.

2) TB  (<10%).

3) Cancer metastases.

 

Clinical Features

 

q       ­ ACTH à Pigmentation: esp of new scars, in exposed  & pressure areas.

q       Glucocorticoid deficiency ® Symptoms are often vague:

Weakness, tiredness, weight loss, anorexia, hypokalemia.

q       Mineralocorticoid deficiency

à  Hypovolemia, Na+ loss, ­ K+ à Postural systolic hypotension.

q       Androgen deficiency à Loss of body hair, impotence, amenorrhea.

 

v    2 Fatal mechanisms:

­ U-Na+          à        ¯ S-Na+           à   ¯ ECV                       à  Hypovolemia  à    ¯ BP                  à        ¯ CO                  à   Prerenal failre          à  DEATH!

 

¯ U-K+            à        ­ S-K+            à  Cardiac arrhythmia  à  DEATH!

 

Investigations

1. A 0900h  P-ACTH level: ­ ACTH &  ¯ Cortisol.

2. The short ACTH (Synachten) stimulation test:     Failure of P-cortisol to rise!

3. Electrolytes & ­ urea.

4. ­ PRA, but S-aldosterone ¯.

5. Adrenal Abs.

6. Chest & abdominal X-rays. (for TB, calcified adrenals).

7. Blood glucose ¯.

 

Treatment

q       Long-term = Replacement glucocorticoid (Cortisol) & mineralocorticoid (Fludrocortisone).

q       TB must be treated if present.

 

N.B. Patient is advised to carry a steroid card / wear a bracelet

 


written by: Khalid Bin Yaroof. FMHS, UAE University.

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