Master the board step 3

Master the Boards: USMLE Step 3
Treatment
1. Phenoxybenzamine (alpha blockade) first to control blood pressure.
Without alpha blockade, patients’ blood pressure can significantly rise
intraoperatively.
2. Propranolol is used after an alpha blocker like phenoxybenzamine.
3. Surgical or laparoscopic resection. Metastatic disease cannot be treated
with surgery.
4. Metastatic disease is treated medically.
Congenital Adrenal Hyperplasia (CAH)
All forms of CAH have the following characteristics:
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Elevated ACTH.
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Low aldosterone and cortisol levels.
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Treatable with prednisone, which inhibits the pituitary.
The different kinds of CAH:
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21 hydroxylase deficiency: This is the most common type.
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Hirsutism caused by increased adrenal androgens and hypotension
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Diagnose with increased 17 hydroxyprogesterone level
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11 hydroxylase deficiency
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Hirsutism caused by increased adrenal androgens and hypertension
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17 hydroxylase deficiency
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Hypertension with low adrenal androgen levels
Hypertension
Virilization
21 No Yes
11 Yes Yes
17 Yes No
Basic Science Correlate
In CAH, hypertension is caused by increased 11-deoxycorticosterone, which
acts like a mineralocorticoid. Because 11- and 17-hydroxylase deficiencies
involve an increased level of 11-deoxycorticosterone, there is hypertension.
Virilization is caused by increased adrenal androgens, DHEA, and
androstenedione.
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