American Board of Surgery Qualifying Exam (ABS QE) Practice Test

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What characterizes 11-hydroxylase deficiency?

  1. Hypotension, low cortisol

  2. Hypertension, high deoxycorticosterone

  3. Hypokalemia, low renin

  4. Hypernatremia, increased estradiol

The correct answer is: Hypertension, high deoxycorticosterone

11-hydroxylase deficiency is a form of congenital adrenal hyperplasia caused by a deficiency in the enzyme that converts 11-deoxycortisol to cortisol and 11-deoxycorticosterone to corticosterone. This deficiency leads to an accumulation of steroid precursors, notably deoxycorticosterone, which has mineralocorticoid activity. The characterization of this condition includes hypertension, which occurs due to the mineralocorticoid effects of high levels of deoxycorticosterone, resulting in sodium retention, increased blood volume, and elevated blood pressure. It is also accompanied by high levels of deoxycorticosterone itself, due to the block in its conversion. Understanding the hormonal imbalances and their physiological effects helps clarify why hypertension and elevated deoxycorticosterone are hallmark findings in this condition. Therefore, the combination of these effects distinctly identifies 11-hydroxylase deficiency from other adrenal disorders.

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