Which of the following is caused by congenital A-17 hydroxylase deficiency:
**Core Concept**
Congenital A-17 hydroxylase deficiency is a rare genetic disorder affecting the adrenal gland's ability to produce cortisol and aldosterone due to a defect in the gene encoding the enzyme 17α-hydroxylase. This enzyme is crucial for the conversion of pregnenolone to 17-hydroxypregnenolone and progesterone to 17-hydroxyprogesterone in the adrenal cortex.
**Why the Correct Answer is Right**
The lack of 17α-hydroxylase activity leads to an accumulation of deoxycorticosterone (DOC) and 11-deoxycortisol, which are potent mineralocorticoids and glucocorticoids, respectively. This results in an overproduction of these hormones, causing hypertension and hypokalemia due to the mineralocorticoid effect, as well as virilization and sexual infantilism due to the glucocorticoid effect.
**Why Each Wrong Option is Incorrect**
* **Option A:** This option is incorrect because the primary defect in congenital A-17 hydroxylase deficiency is not an overproduction of androgens, but rather an overproduction of mineralocorticoids and glucocorticoids.
* **Option B:** This option is incorrect because congenital A-17 hydroxylase deficiency is not associated with an overproduction of catecholamines, but rather an overproduction of mineralocorticoids and glucocorticoids.
* **Option D:** This option is incorrect because congenital A-17 hydroxylase deficiency is not associated with an overproduction of glucagon, but rather an overproduction of mineralocorticoids and glucocorticoids.
**Clinical Pearl / High-Yield Fact**
Congenital A-17 hydroxylase deficiency should be considered in patients with hypertension and hypokalemia, especially in the context of sexual infantilism and virilization. Remember that this condition is often associated with an overproduction of mineralocorticoids and glucocorticoids, leading to hypertension, hypokalemia, and virilization.
**Correct Answer: C. Virilization and hypertension due to overproduction of DOC and 11-deoxycortisol.**