XX newborn with ambiguous genitalia and elevated 17-hydroxyprogesterone levels, most consistent with 21-hydroxylase
Key idea: Three main enzymes that can be deficient in adrenal cortex pathway include (1) 21-hydroxylase (increased testosterone, decreased cortisol, decreased mineralocorticoids) (2) 11-hydroxylase (increased testosterone, decreased cortisol, normal/increased mineralocorticoids because 11-deoxycorticosterone has mineralocorticoid activity (3) 17-hydroxylase (decreased testosterone, decreased cortisol, increased mineralocorticoids)
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